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{
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"results": [
{
"targ_id": "T005004",
"parent_targ_id": "T005",
"full_name": "Cytochrome c oxidase subunit 4 isoform 1, mitochondrial",
"abbrev": "COX IV-1",
"protein_names": "Cytochrome c oxidase subunit 4 isoform 1, mitochondrial (Cytochrome c oxidase polypeptide IV) (Cytochrome c oxidase subunit IV isoform 1) (COX IV-1)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "Oxidoreductase, Transporter",
"Uniport_ID": "P13073",
"Uniprot_name": "COX41_HUMAN",
"EC_numbers": "",
"gene_symbol": "COX4I1",
"gene_synonyms": "COX4, COX4-1",
"gene_synonyms_links": "",
"gene_name": "Cytochrome c oxidase subunit 4I1",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000131143",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Single-pass membrane protein",
"reaction": "",
"targ_desciption": "Component of the cytochrome c oxidase, the last enzyme in themitochondrial electron transport chain which drives oxidativephosphorylation. The respiratory chain contains 3 multisubunitcomplexes succinate dehydrogenase (complex II, CII), ubiquinol-cytochrome c oxidoreductase (cytochrome b-c1 complex, complex III,CIII) and cytochrome c oxidase (complex IV, CIV), that cooperate totransfer electrons derived from NADH and succinate to molecular oxygen,creating an electrochemical gradient over the inner membrane thatdrives transmembrane transport and the ATP synthase. Cytochrome coxidase is the component of the respiratory chain that catalyzes thereduction of oxygen to water. Electrons originating from reducedcytochrome c in the intermembrane space (IMS) are transferred via thedinuclear copper A center (CU(A)) of subunit 2 and heme A of subunbit 1to the active site in subunit 1, a binuclear center (BNC) formed byheme A3 and copper B (CU(B)). The BNC reduces molecular oxygen to 2water molecules using 4 electrons from cytochrome c in the IMS and 4protons from the mitochondrial matrix. {ECO:0000250|UniProtKB:P00424}.",
"references": []
},
{
"targ_id": "T005005",
"parent_targ_id": "T005",
"full_name": "Cytochrome c oxidase subunit 4 isoform 2, mitochondrial",
"abbrev": "COX IV-2",
"protein_names": "Cytochrome c oxidase subunit 4 isoform 2, mitochondrial (Cytochrome c oxidase subunit IV isoform 2) (COX IV-2)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "Oxidoreductase, Transporter",
"Uniport_ID": "Q96KJ9",
"Uniprot_name": "COX42_HUMAN",
"EC_numbers": "",
"gene_symbol": "COX4I2",
"gene_synonyms": "COX4-2, COX4B, COX4L2, COXIV-2, dJ857M17.2",
"gene_synonyms_links": "",
"gene_name": "Cytochrome c oxidase subunit 4I2",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000131055",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Single-pass membrane protein",
"reaction": "",
"targ_desciption": "Component of the cytochrome c oxidase, the last enzyme in themitochondrial electron transport chain which drives oxidativephosphorylation. The respiratory chain contains 3 multisubunitcomplexes succinate dehydrogenase (complex II, CII), ubiquinol-cytochrome c oxidoreductase (cytochrome b-c1 complex, complex III,CIII) and cytochrome c oxidase (complex IV, CIV), that cooperate totransfer electrons derived from NADH and succinate to molecular oxygen,creating an electrochemical gradient over the inner membrane thatdrives transmembrane transport and the ATP synthase. Cytochrome coxidase is the component of the respiratory chain that catalyzes thereduction of oxygen to water. Electrons originating from reducedcytochrome c in the intermembrane space (IMS) are transferred via thedinuclear copper A center (CU(A)) of subunit 2 and heme A of subunbit 1to the active site in subunit 1, a binuclear center (BNC) formed byheme A3 and copper B (CU(B)). The BNC reduces molecular oxygen to 2water molecules using 4 electrons from cytochrome c in the IMS and 4protons from the mitochondrial matrix. {ECO:0000250|UniProtKB:P00424}.",
"references": []
},
{
"targ_id": "T005006",
"parent_targ_id": "T005",
"full_name": "Cytochrome c oxidase subunit 5A, mitochondrial",
"abbrev": "",
"protein_names": "Cytochrome c oxidase subunit 5A, mitochondrial (Cytochrome c oxidase polypeptide Va)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "Transporter",
"Uniport_ID": "P20674",
"Uniprot_name": "COX5A_HUMAN",
"EC_numbers": "",
"gene_symbol": "COX5A",
"gene_synonyms": "",
"gene_synonyms_links": "",
"gene_name": "Cytochrome c oxidase subunit 5A",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000178741",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Peripheral membrane protein; Matrix side",
"reaction": "",
"targ_desciption": "Component of the cytochrome c oxidase, the last enzyme in themitochondrial electron transport chain which drives oxidativephosphorylation. The respiratory chain contains 3 multisubunitcomplexes succinate dehydrogenase (complex II, CII), ubiquinol-cytochrome c oxidoreductase (cytochrome b-c1 complex, complex III,CIII) and cytochrome c oxidase (complex IV, CIV), that cooperate totransfer electrons derived from NADH and succinate to molecular oxygen,creating an electrochemical gradient over the inner membrane thatdrives transmembrane transport and the ATP synthase. Cytochrome coxidase is the component of the respiratory chain that catalyzes thereduction of oxygen to water. Electrons originating from reducedcytochrome c in the intermembrane space (IMS) are transferred via thedinuclear copper A center (CU(A)) of subunit 2 and heme A of subunit 1to the active site in subunit 1, a binuclear center (BNC) formed byheme A3 and copper B (CU(B)). The BNC reduces molecular oxygen to 2water molecules using 4 electrons from cytochrome c in the IMS and 4protons from the mitochondrial matrix. {ECO:0000250|UniProtKB:P00427}.",
"references": []
},
{
"targ_id": "T005007",
"parent_targ_id": "T005",
"full_name": "Cytochrome c oxidase subunit 6A1, mitochondrial",
"abbrev": "COX VIa-L",
"protein_names": "Cytochrome c oxidase subunit 6A1, mitochondrial (Cytochrome c oxidase polypeptide VIa-liver) (Cytochrome c oxidase subunit VIA-liver) (COX VIa-L)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "Oxidoreductase",
"Uniport_ID": "P12074",
"Uniprot_name": "CX6A1_HUMAN",
"EC_numbers": "",
"gene_symbol": "COX6A1",
"gene_synonyms": "COX6A",
"gene_synonyms_links": "",
"gene_name": "Cytochrome c oxidase subunit 6A1",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000111775",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Single-pass membrane protein",
"reaction": "",
"targ_desciption": "Component of the cytochrome c oxidase, the last enzyme in themitochondrial electron transport chain which drives oxidativephosphorylation. The respiratory chain contains 3 multisubunitcomplexes succinate dehydrogenase (complex II, CII), ubiquinol-cytochrome c oxidoreductase (cytochrome b-c1 complex, complex III,CIII) and cytochrome c oxidase (complex IV, CIV), that cooperate totransfer electrons derived from NADH and succinate to molecular oxygen,creating an electrochemical gradient over the inner membrane thatdrives transmembrane transport and the ATP synthase. Cytochrome coxidase is the component of the respiratory chain that catalyzes thereduction of oxygen to water. Electrons originating from reducedcytochrome c in the intermembrane space (IMS) are transferred via thedinuclear copper A center (CU(A)) of subunit 2 and heme A of subunit 1to the active site in subunit 1, a binuclear center (BNC) formed byheme A3 and copper B (CU(B)). The BNC reduces molecular oxygen to 2water molecules unsing 4 electrons from cytochrome c in the IMS and 4protons from the mitochondrial matrix. {ECO:0000250|UniProtKB:P32799}.",
"references": []
},
{
"targ_id": "T005008",
"parent_targ_id": "T005",
"full_name": "Cytochrome c oxidase subunit 6B1",
"abbrev": "COX VIb-1",
"protein_names": "Cytochrome c oxidase subunit 6B1 (Cytochrome c oxidase subunit VIb isoform 1) (COX VIb-1)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "P14854",
"Uniprot_name": "CX6B1_HUMAN",
"EC_numbers": "",
"gene_symbol": "COX6B1",
"gene_synonyms": "COX6B, COXG",
"gene_synonyms_links": "",
"gene_name": "Cytochrome c oxidase subunit 6B1",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000126267",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Peripheral membrane protein; Intermembrane side",
"reaction": "",
"targ_desciption": "Component of the cytochrome c oxidase, the last enzyme in themitochondrial electron transport chain which drives oxidativephosphorylation. The respiratory chain contains 3 multisubunitcomplexes succinate dehydrogenase (complex II, CII), ubiquinol-cytochrome c oxidoreductase (cytochrome b-c1 complex, complex III,CIII) and cytochrome c oxidase (complex IV, CIV), that cooperate totransfer electrons derived from NADH and succinate to molecular oxygen,creating an electrochemical gradient over the inner membrane thatdrives transmembrane transport and the ATP synthase. Cytochrome coxidase is the component of the respiratory chain that catalyzes thereduction of oxygen to water. Electrons originating from reducedcytochrome c in the intermembrane space (IMS) are transferred via thedinuclear copper A center (CU(A)) of subunit 2 and heme A of subunit 1to the active site in subunit 1, a binuclear center (BNC) formed byheme A3 and copper B (CU(B)). The BNC reduces molecular oxygen to 2water molecules using 4 electrons from cytochrome c in the IMS and 4protons from the mitochondrial matrix. {ECO:0000250|UniProtKB:Q01519}.",
"references": []
},
{
"targ_id": "T005009",
"parent_targ_id": "T005",
"full_name": "Cytochrome c oxidase subunit 7B, mitochondrial",
"abbrev": "",
"protein_names": "Cytochrome c oxidase subunit 7B, mitochondrial (Cytochrome c oxidase polypeptide VIIb)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "P24311",
"Uniprot_name": "COX7B_HUMAN",
"EC_numbers": "",
"gene_symbol": "COX7B",
"gene_synonyms": "",
"gene_synonyms_links": "",
"gene_name": "Cytochrome c oxidase subunit 7B",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000131174",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Single-pass membrane protein",
"reaction": "",
"targ_desciption": "Component of the cytochrome c oxidase, the last enzyme in themitochondrial electron transport chain which drives oxidativephosphorylation. The respiratory chain contains 3 multisubunitcomplexes succinate dehydrogenase (complex II, CII), ubiquinol-cytochrome c oxidoreductase (cytochrome b-c1 complex, complex III,CIII) and cytochrome c oxidase (complex IV, CIV), that cooperate totransfer electrons derived from NADH and succinate to molecular oxygen,creating an electrochemical gradient over the inner membrane thatdrives transmembrane transport and the ATP synthase. Cytochrome coxidase is the component of the respiratory chain that catalyzes thereduction of oxygen to water. Electrons originating from reducedcytochrome c in the intermembrane space (IMS) are transferred via thedinuclear copper A center (CU(A)) of subunit 2 and heme A of subunit 1to the active site in subunit 1, a binuclear center (BNC) formed byheme A3 and copper B (CU(B)). The BNC reduces molecular oxygen to 2water molecules using 4 electrons from cytochrome c in the IMS and 4protons from the mitochondrial matrix (By similarity). Plays a role inproper central nervous system (CNS) development in vertebrates(PubMed:23122588). {ECO:0000250|UniProtKB:P13183,ECO:0000269|PubMed:23122588}.",
"references": []
},
{
"targ_id": "T005010",
"parent_targ_id": "T005",
"full_name": "Cytochrome c oxidase subunit 8A, mitochondrial",
"abbrev": "Cytochrome c oxidase subunit 8-2",
"protein_names": "Cytochrome c oxidase subunit 8A, mitochondrial (Cytochrome c oxidase polypeptide VIII-liver/heart) (Cytochrome c oxidase subunit 8-2)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "Transporter",
"Uniport_ID": "P10176",
"Uniprot_name": "COX8A_HUMAN",
"EC_numbers": "",
"gene_symbol": "COX8A",
"gene_synonyms": "COX, COX8, COX8-2, COX8L, VIII, VIII-L",
"gene_synonyms_links": "",
"gene_name": "Cytochrome c oxidase subunit 8A",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000176340",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Single-pass membrane protein",
"reaction": "",
"targ_desciption": "Component of the cytochrome c oxidase, the last enzyme in themitochondrial electron transport chain which drives oxidativephosphorylation. The respiratory chain contains 3 multisubunitcomplexes succinate dehydrogenase (complex II, CII), ubiquinol-cytochrome c oxidoreductase (cytochrome b-c1 complex, complex III,CIII) and cytochrome c oxidase (complex IV, CIV), that cooperate totransfer electrons derived from NADH and succinate to molecular oxygen,creating an electrochemical gradient over the inner membrane thatdrives transmembrane transport and the ATP synthase. Cytochrome coxidase is the component of the respiratory chain that catalyzes thereduction of oxygen to water. Electrons originating from reducedcytochrome c in the intermembrane space (IMS) are transferred via thedinuclear copper A center (CU(A)) of subunit 2 and heme A of subunit 1to the active site in subunit 1, a binuclear center (BNC) formed byheme A3 and copper B (CU(B)). The BNC reduces molecular oxygen to 2water molecules using 4 electrons from cytochrome c in the IMS and 4protons from the mitochondrial matrix. {ECO:0000250|UniProtKB:P10175}.",
"references": []
},
{
"targ_id": "T005011",
"parent_targ_id": "T005",
"full_name": "Cytochrome c oxidase subunit NDUFA4",
"abbrev": "CI-MLRQ",
"protein_names": "Cytochrome c oxidase subunit NDUFA4 (Complex I-MLRQ) (CI-MLRQ) (NADH-ubiquinone oxidoreductase MLRQ subunit)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "O00483",
"Uniprot_name": "NDUA4_HUMAN",
"EC_numbers": "",
"gene_symbol": "NDUFA4",
"gene_synonyms": "CI-9k, MLRQ",
"gene_synonyms_links": "",
"gene_name": "NDUFA4, mitochondrial complex associated",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000189043",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Single-pass membrane protein",
"reaction": "",
"targ_desciption": "Component of the cytochrome c oxidase, the last enzyme in themitochondrial electron transport chain which drives oxidativephosphorylation. The respiratory chain contains 3 multisubunitcomplexes succinate dehydrogenase (complex II, CII), ubiquinol-cytochrome c oxidoreductase (cytochrome b-c1 complex, complex III,CIII) and cytochrome c oxidase (complex IV, CIV), that cooperate totransfer electrons derived from NADH and succinate to molecular oxygen,creating an electrochemical gradient over the inner membrane thatdrives transmembrane transport and the ATP synthase. Cytochrome coxidase is the component of the respiratory chain that catalyzes thereduction of oxygen to water. Electrons originating from reducedcytochrome c in the intermembrane space (IMS) are transferred via thedinuclear copper A center (CU(A)) of subunit 2 and heme A of subunit 1to the active site in subunit 1, a binuclear center (BNC) formed byheme A3 and copper B (CU(B)). The BNC reduces molecular oxygen to 2water molecules unsing 4 electrons from cytochrome c in the IMS and 4protons from the mitochondrial matrix (PubMed:22902835). NDUFA4 isrequired for complex IV maintenance (PubMed:22902835).{ECO:0000269|PubMed:22902835}.",
"references": []
},
{
"targ_id": "T005012",
"parent_targ_id": "T005",
"full_name": "Cytochrome c oxidase assembly factor 5",
"abbrev": "",
"protein_names": "Cytochrome c oxidase assembly factor 5",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "Q86WW8",
"Uniprot_name": "COA5_HUMAN",
"EC_numbers": "",
"gene_symbol": "COA5",
"gene_synonyms": "C2orf64, FLJ27524, MGC52110, Pet191",
"gene_synonyms_links": "",
"gene_name": "Cytochrome c oxidase assembly factor 5",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000183513",
"omim_id": "",
"genecard_link": "",
"locs": "",
"reaction": "",
"targ_desciption": "Involved in an early step of the mitochondrial complex IVassembly process. {ECO:0000269|PubMed:21457908}.",
"references": []
},
{
"targ_id": "T005013",
"parent_targ_id": "T005",
"full_name": "Cytochrome c oxidase assembly factor 6 homolog",
"abbrev": "",
"protein_names": "Cytochrome c oxidase assembly factor 6 homolog",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "Q5JTJ3",
"Uniprot_name": "COA6_HUMAN",
"EC_numbers": "",
"gene_symbol": "COA6",
"gene_synonyms": "C1orf31",
"gene_synonyms_links": "",
"gene_name": "Cytochrome c oxidase assembly factor 6",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000168275",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion intermembrane space",
"reaction": "",
"targ_desciption": "Involved in the maturation of the mitochondrial respiratorychain complex IV subunit MT-CO2/COX2. Thereby, may regulate early stepsof complex IV assembly. Mitochondrial respiratory chain complex IV orcytochrome c oxidase is the component of the respiratory chain thatcatalyzes the transfer of electrons from intermembrane space cytochromec to molecular oxygen in the matrix and as a consequence contributes tothe proton gradient involved in mitochondrial ATP synthesis. May alsobe required for efficient formation of respiratory supercomplexescomprised of complexes III and IV. {ECO:0000269|PubMed:24549041,ECO:0000269|PubMed:25959673, ECO:0000269|PubMed:26160915}.",
"references": []
},
{
"targ_id": "T005014",
"parent_targ_id": "T005",
"full_name": "Cytochrome c oxidase assembly factor 7",
"abbrev": "",
"protein_names": "Cytochrome c oxidase assembly factor 7 (Beta-lactamase hcp-like protein) (Respiratory chain assembly factor 1) (Sel1 repeat-containing protein 1)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "Q96BR5",
"Uniprot_name": "COA7_HUMAN",
"EC_numbers": "",
"gene_symbol": "COA7",
"gene_synonyms": "C1orf163, FLJ12439, RESA1, SELRC1",
"gene_synonyms_links": "",
"gene_name": "Cytochrome c oxidase assembly factor 7 (putative)",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000162377",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion intermembrane space",
"reaction": "",
"targ_desciption": "Required for assembly of mitochondrial respiratory chaincomplex I and complex IV. {ECO:0000269|PubMed:24333015}.",
"references": []
},
{
"targ_id": "T005015",
"parent_targ_id": "T005",
"full_name": "Cytochrome c oxidase assembly protein COX14",
"abbrev": "",
"protein_names": "Cytochrome c oxidase assembly protein COX14",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "Q96I36",
"Uniprot_name": "COX14_HUMAN",
"EC_numbers": "",
"gene_symbol": "COX14",
"gene_synonyms": "C12orf62, MGC14288",
"gene_synonyms_links": "",
"gene_name": "COX14, cytochrome c oxidase assembly factor",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000178449",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion membrane; Single-pass membrane protein",
"reaction": "",
"targ_desciption": "Core component of the MITRAC (mitochondrial translationregulation assembly intermediate of cytochrome c oxidase complex)complex, that regulates cytochrome c oxidase assembly. Requires forcoordination of the early steps of cytochrome c oxidase assembly withthe synthesis of MT-CO1. {ECO:0000269|PubMed:22243966,ECO:0000269|PubMed:22356826}.",
"references": []
},
{
"targ_id": "T005016",
"parent_targ_id": "T005",
"full_name": "Cytochrome c oxidase assembly protein COX20, mitochondrial",
"abbrev": "",
"protein_names": "Cytochrome c oxidase assembly protein COX20, mitochondrial",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "Q5RI15",
"Uniprot_name": "COX20_HUMAN",
"EC_numbers": "",
"gene_symbol": "COX20",
"gene_synonyms": "FAM36A, FLJ43269",
"gene_synonyms_links": "",
"gene_name": "COX20, cytochrome c oxidase assembly factor",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000203667",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Multi-pass membrane protein",
"reaction": "",
"targ_desciption": "Essential for the assembly of the mitochondrial respiratorychain complex IV (CIV), also known as cytochrome c oxidase(PubMed:23125284). Acts as a chaperone in the early steps of cytochromec oxidase subunit II (MT-CO2/COX2) maturation, stabilizing the newlysynthesized protein and presenting it to metallochaperones SCO1/2 whichin turn facilitates the incorporation of the mature MT-CO2/COX2 intothe assembling CIV holoenzyme (PubMed:24403053).{ECO:0000269|PubMed:23125284, ECO:0000269|PubMed:24403053}.",
"references": []
},
{
"targ_id": "T005017",
"parent_targ_id": "T005",
"full_name": "Cytochrome c oxidase subunit 5B, mitochondrial",
"abbrev": "Cytochrome c oxidase polypeptide Vb",
"protein_names": "Cytochrome c oxidase subunit 5B, mitochondrial (Cytochrome c oxidase polypeptide Vb)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "P10606",
"Uniprot_name": "COX5B_HUMAN",
"EC_numbers": "",
"gene_symbol": "COX5B",
"gene_synonyms": "",
"gene_synonyms_links": "",
"gene_name": "Cytochrome c oxidase subunit 5B",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000135940",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Peripheral membrane protein; Matrix side",
"reaction": "",
"targ_desciption": "Component of the cytochrome c oxidase, the last enzyme in themitochondrial electron transport chain which drives oxidativephosphorylation. The respiratory chain contains 3 multisubunitcomplexes succinate dehydrogenase (complex II, CII), ubiquinol-cytochrome c oxidoreductase (cytochrome b-c1 complex, complex III,CIII) and cytochrome c oxidase (complex IV, CIV), that cooperate totransfer electrons derived from NADH and succinate to molecular oxygen,creating an electrochemical gradient over the inner membrane thatdrives transmembrane transport and the ATP synthase. Cytochrome coxidase is the component of the respiratory chain that catalyzes thereduction of oxygen to water. Electrons originating from reducedcytochrome c in the intermembrane space (IMS) are transferred via thedinuclear copper A center (CU(A)) of subunit 2 and heme A of subunit 1to the active site in subunit 1, a binuclear center (BNC) formed byheme A3 and copper B (CU(B)). The BNC reduces molecular oxygen to 2water molecules using 4 electrons from cytochrome c in the IMS and 4protons from the mitochondrial matrix. {ECO:0000250|UniProtKB:P04037}.",
"references": []
},
{
"targ_id": "T005018",
"parent_targ_id": "T005",
"full_name": "Cytochrome c oxidase subunit 6A2, mitochondrial",
"abbrev": "COXVIAH",
"protein_names": "Cytochrome c oxidase subunit 6A2, mitochondrial (Cytochrome c oxidase polypeptide VIa-heart) (COXVIAH) (Cytochrome c oxidase subunit VIA-muscle) (COX VIa-M)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "Q02221",
"Uniprot_name": "CX6A2_HUMAN",
"EC_numbers": "",
"gene_symbol": "COX6A2",
"gene_synonyms": "",
"gene_synonyms_links": "",
"gene_name": "Cytochrome c oxidase subunit 6A2",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000156885",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Single-pass membrane protein",
"reaction": "",
"targ_desciption": "Component of the cytochrome c oxidase, the last enzyme in themitochondrial electron transport chain which drives oxidativephosphorylation. The respiratory chain contains 3 multisubunitcomplexes succinate dehydrogenase (complex II, CII), ubiquinol-cytochrome c oxidoreductase (cytochrome b-c1 complex, complex III,CIII) and cytochrome c oxidase (complex IV, CIV), that cooperate totransfer electrons derived from NADH and succinate to molecular oxygen,creating an electrochemical gradient over the inner membrane thatdrives transmembrane transport and the ATP synthase. Cytochrome coxidase is the component of the respiratory chain that catalyzes thereduction of oxygen to water. Electrons originating from reducedcytochrome c in the intermembrane space (IMS) are transferred via thedinuclear copper A center (CU(A)) of subunit 2 and heme A of subunit 1to the active site in subunit 1, a binuclear center (BNC) formed byheme A3 and copper B (CU(B)). The BNC reduces molecular oxygen to 2water molecules unsing 4 electrons from cytochrome c in the IMS and 4protons from the mitochondrial matrix. {ECO:0000250|UniProtKB:P32799}.",
"references": []
},
{
"targ_id": "T005019",
"parent_targ_id": "T005",
"full_name": "Cytochrome c oxidase subunit 6C",
"abbrev": "",
"protein_names": "Cytochrome c oxidase subunit 6C (Cytochrome c oxidase polypeptide VIc)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "Oxidoreductase",
"Uniport_ID": "P09669",
"Uniprot_name": "COX6C_HUMAN",
"EC_numbers": "",
"gene_symbol": "COX6C",
"gene_synonyms": "",
"gene_synonyms_links": "",
"gene_name": "Cytochrome c oxidase subunit 6C",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000164919",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Single-pass membrane protein",
"reaction": "",
"targ_desciption": "Component of the cytochrome c oxidase, the last enzyme in themitochondrial electron transport chain which drives oxidativephosphorylation. The respiratory chain contains 3 multisubunitcomplexes succinate dehydrogenase (complex II, CII), ubiquinol-cytochrome c oxidoreductase (cytochrome b-c1 complex, complex III,CIII) and cytochrome c oxidase (complex IV, CIV), that cooperate totransfer electrons derived from NADH and succinate to molecular oxygen,creating an electrochemical gradient over the inner membrane thatdrives transmembrane transport and the ATP synthase. Cytochrome coxidase is the component of the respiratory chain that catalyzes thereduction of oxygen to water. Electrons originating from reducedcytochrome c in the intermembrane space (IMS) are transferred via thedinuclear copper A center (CU(A)) of subunit 2 and heme A of subunit 1to the active site in subunit 1, a binuclear center (BNC) formed byheme A3 and copper B (CU(B)). The BNC reduces molecular oxygen to 2water molecules using 4 electrons from cytochrome c in the IMS and 4protons from the mitochondrial matrix. {ECO:0000250|UniProtKB:P04038}.",
"references": []
},
{
"targ_id": "T005020",
"parent_targ_id": "T005",
"full_name": "Cytochrome c oxidase subunit 7A1, mitochondrial",
"abbrev": "",
"protein_names": "Cytochrome c oxidase subunit 7A1, mitochondrial (Cytochrome c oxidase subunit VIIa-heart) (Cytochrome c oxidase subunit VIIa-H) (Cytochrome c oxidase subunit VIIa-muscle) (Cytochrome c oxidase subunit VIIa-M)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "P24310",
"Uniprot_name": "CX7A1_HUMAN",
"EC_numbers": "",
"gene_symbol": "COX7A1",
"gene_synonyms": "COX7A, COX7AH",
"gene_synonyms_links": "",
"gene_name": "Cytochrome c oxidase subunit 7A1",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000161281",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Single-pass membrane protein",
"reaction": "",
"targ_desciption": "Component of the cytochrome c oxidase, the last enzyme in themitochondrial electron transport chain which drives oxidativephosphorylation. The respiratory chain contains 3 multisubunitcomplexes succinate dehydrogenase (complex II, CII), ubiquinol-cytochrome c oxidoreductase (cytochrome b-c1 complex, complex III,CIII) and cytochrome c oxidase (complex IV, CIV), that cooperate totransfer electrons derived from NADH and succinate to molecular oxygen,creating an electrochemical gradient over the inner membrane thatdrives transmembrane transport and the ATP synthase. Cytochrome coxidase is the component of the respiratory chain that catalyzes thereduction of oxygen to water. Electrons originating from reducedcytochrome c in the intermembrane space (IMS) are transferred via thedinuclear copper A center (CU(A)) of subunit 2 and heme A of subunit 1to the active site in subunit 1, a binuclear center (BNC) formed byheme A3 and copper B (CU(B)). The BNC reduces molecular oxygen to 2water molecules using 4 electrons from cytochrome c in the IMS and 4protons from the mitochondrial matrix. {ECO:0000250|UniProtKB:P10174}.",
"references": []
},
{
"targ_id": "T005021",
"parent_targ_id": "T005",
"full_name": "Cytochrome c oxidase subunit 7C, mitochondrial",
"abbrev": "",
"protein_names": "Cytochrome c oxidase subunit 7C, mitochondrial (Cytochrome c oxidase polypeptide VIIc)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "Oxidoreductase",
"Uniport_ID": "P15954",
"Uniprot_name": "COX7C_HUMAN",
"EC_numbers": "",
"gene_symbol": "COX7C",
"gene_synonyms": "",
"gene_synonyms_links": "",
"gene_name": "Cytochrome c oxidase subunit 7C",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000127184",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Single-pass membrane protein",
"reaction": "",
"targ_desciption": "Component of the cytochrome c oxidase, the last enzyme in themitochondrial electron transport chain which drives oxidativephosphorylation. The respiratory chain contains 3 multisubunitcomplexes succinate dehydrogenase (complex II, CII), ubiquinol-cytochrome c oxidoreductase (cytochrome b-c1 complex, complex III,CIII) and cytochrome c oxidase (complex IV, CIV), that cooperate totransfer electrons derived from NADH and succinate to molecular oxygen,creating an electrochemical gradient over the inner membrane thatdrives transmembrane transport and the ATP synthase. Cytochrome coxidase is the component of the respiratory chain that catalyzes thereduction of oxygen to water. Electrons originating from reducedcytochrome c in the intermembrane space (IMS) are transferred via thedinuclear copper A center (CU(A)) of subunit 2 and heme A of subunit 1to the active site in subunit 1, a binuclear center (BNC) formed byheme A3 and copper B (CU(B)). The BNC reduces molecular oxygen to 2water molecules using 4 electrons from cytochrome c in the IMS and 4protons from the mitochondrial matrix. {ECO:0000250|UniProtKB:P04039}.",
"references": []
},
{
"targ_id": "T006",
"parent_targ_id": "T",
"full_name": "Surfeit locus protein 1",
"abbrev": "",
"protein_names": "Surfeit locus protein 1",
"related_func_ids": "F020103",
"category": "protein",
"subcategories": "",
"Uniport_ID": "Q15526",
"Uniprot_name": "SURF1_HUMAN",
"EC_numbers": "",
"gene_symbol": "SURF1",
"gene_synonyms": "",
"gene_synonyms_links": "",
"gene_name": "SURF1, cytochrome c oxidase assembly factor",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000148290",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Multi-pass membrane protein",
"reaction": "",
"targ_desciption": "Component of the MITRAC (mitochondrial translation regulationassembly intermediate of cytochrome c oxidase complex) complex, thatregulates cytochrome c oxidase assembly. {ECO:0000269|PubMed:24027061,ECO:0000269|PubMed:9843204, ECO:0000305|PubMed:26321642}.",
"references": []
},
{
"targ_id": "T007",
"parent_targ_id": "T",
"full_name": "Protein SCO1 homolog, mitochondrial",
"abbrev": "",
"protein_names": "Protein SCO1 homolog, mitochondrial",
"related_func_ids": "F020103",
"category": "protein",
"subcategories": "Chaperone",
"Uniport_ID": "O75880",
"Uniprot_name": "SCO1_HUMAN",
"EC_numbers": "",
"gene_symbol": "SCO1",
"gene_synonyms": "SCOD1",
"gene_synonyms_links": "",
"gene_name": "SCO1, cytochrome c oxidase assembly protein",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000133028",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion; Mitochondrion inner membrane; Single-pass membrane protein",
"reaction": "",
"targ_desciption": "Copper metallochaperone essential for the maturation ofcytochrome c oxidase subunit II (MT-CO2/COX2). Not required for thesynthesis of MT-CO2/COX2 but plays a crucial role in stabilizing MT-CO2/COX2 during its subsequent maturation. Involved in transportingcopper to the Cu(A) site on MT-CO2/COX2 (PubMed:15659396,PubMed:16735468, PubMed:17189203, PubMed:19336478, PubMed:15229189).Plays an important role in the regulation of copper homeostasis bycontrolling the abundance and cell membrane localization of coppertransporter CTR1 (By similarity). {ECO:0000250|UniProtKB:Q5SUC9,ECO:0000269|PubMed:15229189, ECO:0000269|PubMed:15659396,ECO:0000269|PubMed:16735468, ECO:0000269|PubMed:17189203,ECO:0000269|PubMed:19336478}.",
"references": []
},
{
"targ_id": "T008",
"parent_targ_id": "T",
"full_name": "Protein SCO2 homolog, mitochondrial",
"abbrev": "",
"protein_names": "Protein SCO2 homolog, mitochondrial",
"related_func_ids": "F020103",
"category": "protein",
"subcategories": "Chaperone",
"Uniport_ID": "O43819",
"Uniprot_name": "SCO2_HUMAN",
"EC_numbers": "",
"gene_symbol": "SCO2",
"gene_synonyms": "MYP6, SCO1L",
"gene_synonyms_links": "",
"gene_name": "SCO2, cytochrome c oxidase assembly protein",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000130489",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Single-pass membrane protein",
"reaction": "",
"targ_desciption": "Copper metallochaperone essential for the synthesis andmaturation of cytochrome c oxidase subunit II (MT-CO2/COX2). Involvedin transporting copper to the Cu(A) site on MT-CO2/COX2(PubMed:15229189, PubMed:17189203). Also acts as a thiol-disulfideoxidoreductase to regulate the redox state of the cysteines in SCO1during maturation of MT-CO2/COX2 (PubMed:19336478).{ECO:0000269|PubMed:15229189, ECO:0000269|PubMed:17189203,ECO:0000269|PubMed:19336478}.",
"references": []
},
{
"targ_id": "T009",
"parent_targ_id": "T",
"full_name": "Protoheme IX farnesyltransferase, mitochondrial",
"abbrev": "Heme O synthase",
"protein_names": "Protoheme IX farnesyltransferase, mitochondrial (EC 2.5.1.141) (Heme O synthase)",
"related_func_ids": "F020103; F021205; F030804",
"category": "enzyme",
"subcategories": "Transferase",
"Uniport_ID": "Q12887",
"Uniprot_name": "COX10_HUMAN",
"EC_numbers": "2.5.1.141",
"gene_symbol": "COX10",
"gene_synonyms": "",
"gene_synonyms_links": "",
"gene_name": "COX10, heme A:farnesyltransferase cytochrome c oxidase assembly factor",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000006695",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion membrane; Multi-pass membrane protein",
"reaction": "(2E,6E)-farnesyl diphosphate + H2O + heme b = diphosphate +Fe(II)-heme o",
"targ_desciption": "Converts protoheme IX and farnesyl diphosphate to heme O.{ECO:0000250}.",
"references": []
},
{
"targ_id": "T010",
"parent_targ_id": "T",
"full_name": "Cytochrome c oxidase assembly protein COX15 homolog",
"abbrev": "",
"protein_names": "Cytochrome c oxidase assembly protein COX15 homolog",
"related_func_ids": "F020103; F021205",
"category": "protein",
"subcategories": "",
"Uniport_ID": "Q7KZN9",
"Uniprot_name": "COX15_HUMAN",
"EC_numbers": "",
"gene_symbol": "COX15",
"gene_synonyms": "CEMCOX2",
"gene_synonyms_links": "",
"gene_name": "COX15, cytochrome c oxidase assembly homolog",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000014919",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion membrane; Multi-pass membrane protein",
"reaction": "",
"targ_desciption": "May be involved in the biosynthesis of heme A.{ECO:0000269|PubMed:12474143}.",
"references": []
},
{
"targ_id": "T011",
"parent_targ_id": "T",
"full_name": "Succinyl-CoA:3-ketoacid coenzyme A transferase 1, mitochondrial",
"abbrev": "SCOT-s",
"protein_names": "Succinyl-CoA:3-ketoacid coenzyme A transferase 1, mitochondrial (EC 2.8.3.5) (3-oxoacid CoA-transferase 1) (Somatic-type succinyl-CoA:3-oxoacid CoA-transferase) (SCOT-s)",
"related_func_ids": "F02060202",
"category": "enzyme",
"subcategories": "Transferase",
"Uniport_ID": "P55809",
"Uniprot_name": "SCOT1_HUMAN",
"EC_numbers": "2.8.3.5",
"gene_symbol": "OXCT1",
"gene_synonyms": "OXCT, SCOT",
"gene_synonyms_links": "",
"gene_name": "3-oxoacid CoA-transferase 1",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000083720",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion matrix",
"reaction": "a 3-oxo acid + succinyl-CoA = a 3-oxoacyl-CoA + succinate",
"targ_desciption": "Key enzyme for ketone body catabolism. Transfers the CoAmoiety from succinate to acetoacetate. Formation of the enzyme-CoAintermediate proceeds via an unstable anhydride species formed betweenthe carboxylate groups of the enzyme and substrate.",
"references": []
},
{
"targ_id": "T012",
"parent_targ_id": "T",
"full_name": "FAST kinase domain-containing protein 2, mitochondrial",
"abbrev": "",
"protein_names": "FAST kinase domain-containing protein 2, mitochondrial",
"related_func_ids": "F060302; F0604",
"category": "protein",
"subcategories": "",
"Uniport_ID": "Q9NYY8",
"Uniprot_name": "FAKD2_HUMAN",
"EC_numbers": "",
"gene_symbol": "FASTKD2",
"gene_synonyms": "KIAA0971",
"gene_synonyms_links": "",
"gene_name": "FAST kinase domains 2",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000118246",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion matrix, mitochondrion nucleoid",
"reaction": "",
"targ_desciption": "Plays an important role in assembly of the mitochondriallarge ribosomal subunit (PubMed:25683715). As a component of afunctional protein-RNA module, consisting of RCC1L, NGRN, RPUSD3,RPUSD4, TRUB2, FASTKD2 and 16S mitochondrial ribosomal RNA (16S mt-rRNA), controls 16S mt-rRNA abundance and is required for intra-mitochondrial translation (PubMed:27667664).{ECO:0000269|PubMed:25683715, ECO:0000269|PubMed:27667664}.",
"references": []
},
{
"targ_id": "T013",
"parent_targ_id": "T",
"full_name": "Protein PET100 homolog, mitochondrial",
"abbrev": "",
"protein_names": "Protein PET100 homolog, mitochondrial",
"related_func_ids": "F020103",
"category": "protein",
"subcategories": "",
"Uniport_ID": "P0DJ07",
"Uniprot_name": "PT100_HUMAN",
"EC_numbers": "",
"gene_symbol": "PET100",
"gene_synonyms": "C19orf79",
"gene_synonyms_links": "",
"gene_name": "PET100 homolog",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000229833",
"omim_id": "",
"genecard_link": "",
"locs": "Membrane; Single-pass membrane protein; Mitochondrion",
"reaction": "",
"targ_desciption": "",
"references": []
},
{
"targ_id": "T014",
"parent_targ_id": "T",
"full_name": "Protein PET117 homolog, mitochondrial",
"abbrev": "",
"protein_names": "Protein PET117 homolog, mitochondrial",
"related_func_ids": "F020103",
"category": "protein",
"subcategories": "",
"Uniport_ID": "Q6UWS5",
"Uniprot_name": "PT117_HUMAN",
"EC_numbers": "",
"gene_symbol": "PET117",
"gene_synonyms": "CSRP2BP",
"gene_synonyms_links": "",
"gene_name": "PET117 homolog",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000232838",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion",
"reaction": "",
"targ_desciption": "",
"references": []
},
{
"targ_id": "T015",
"parent_targ_id": "T",
"full_name": "Centrosomal protein of 89 kDa",
"abbrev": "Cep89",
"protein_names": "Centrosomal protein of 89 kDa (Cep89) (Centrosomal protein 123) (Cep123) (Coiled-coil domain-containing protein 123)",
"related_func_ids": "F03",
"category": "protein",
"subcategories": "",
"Uniport_ID": "Q96ST8",
"Uniprot_name": "CEP89_HUMAN",
"EC_numbers": "",
"gene_symbol": "CEP89",
"gene_synonyms": "CCDC123, FLJ14640",
"gene_synonyms_links": "",
"gene_name": "Centrosomal protein 89",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000121289",
"omim_id": "",
"genecard_link": "",
"locs": "Cytoplasm, cytosol; Cytoplasm, cytoskeleton, microtubule organizing center, centrosome; Cytoplasm, cytoskeleton, spindle pole; Cytoplasm, cytoskeleton, microtubule organizing center, centrosome,centriole; Mitochondrion intermembrane space",
"reaction": "",
"targ_desciption": "Required for ciliogenesis. Also plays a role in mitochondrialmetabolism where it may modulate complex IV activity.{ECO:0000269|PubMed:23348840, ECO:0000269|PubMed:23575228}.",
"references": []
},
{
"targ_id": "T016",
"parent_targ_id": "T",
"full_name": "oxygen",
"abbrev": "oxygen",
"protein_names": "",
"related_func_ids": "",
"category": "molecule",
"subcategories": "",
"Uniport_ID": "",
"Uniprot_name": "",
"EC_numbers": "",
"gene_symbol": "",
"gene_synonyms": "",
"gene_synonyms_links": "",
"gene_name": "",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "",
"omim_id": "",
"genecard_link": "",
"locs": "",
"reaction": "",
"targ_desciption": "",
"references": [
"RC01164",
"RC01165",
"RC01166",
"RC01167",
"RC01168",
"RC01169"
]
},
{
"targ_id": "T018",
"parent_targ_id": "T",
"full_name": "copper",
"abbrev": "copper",
"protein_names": "",
"related_func_ids": "",
"category": "molecule",
"subcategories": "",
"Uniport_ID": "",
"Uniprot_name": "",
"EC_numbers": "",
"gene_symbol": "",
"gene_synonyms": "",
"gene_synonyms_links": "",
"gene_name": "",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "",
"omim_id": "",
"genecard_link": "",
"locs": "",
"reaction": "",
"targ_desciption": "",
"references": [
"RC01179",
"RC01181"
]
},
{
"targ_id": "T019",
"parent_targ_id": "T",
"full_name": "ATP synthase",
"abbrev": "Complex V",
"protein_names": "",
"related_func_ids": "F020103",
"category": "enzyme",
"subcategories": "",
"Uniport_ID": "",
"Uniprot_name": "",
"EC_numbers": "7.1.2.2",
"gene_symbol": "",
"gene_synonyms": "",
"gene_synonyms_links": "",
"gene_name": "",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "",
"omim_id": "",
"genecard_link": "",
"locs": "",
"reaction": "ATP + 4 H(+)(in) + H2O = ADP + 5 H(+)(out) + phosphate",
"targ_desciption": "",
"references": [
"RC00110",
"RC00111",
"RC00116",
"RC00121",
"RC00126",
"RC00131",
"RC00136",
"RC00141",
"RC00146",
"RC00151",
"RC00156",
"RC00161",
"RC00166",
"RC00171",
"RC00176",
"RC00181",
"RC00186",
"RC00191",
"RC00196",
"RC00201",
"RC00206",
"RC00211",
"RC00217",
"RC00220",
"RC00221",
"RC00933",
"RC00944",
"RC00957",
"RC00968",
"RC01417",
"RC01418",
"RC01419",
"RC01420",
"RC01421",
"RC01422",
"RC01423",
"RC01424",
"RC01425",
"RC01426",
"RC01427",
"RC01428",
"RC01429",
"RC01430",
"RC01431",
"RC01432",
"RC01433",
"RC01434",
"RC01435",
"RC01436",
"RC01437",
"RC01438",
"RC01439",
"RC01440",
"RC01441",
"RC01442",
"RC01443",
"RC01444",
"RC01445",
"RC01446",
"RC01447",
"RC01448",
"RC01449",
"RC01450",
"RC01451",
"RC01452",
"RC01453",
"RC01454",
"RC01455",
"RC01456",
"RC01457",
"RC01458",
"RC01459",
"RC01460",
"RC01461",
"RC01462",
"RC01463",
"RC01464",
"RC01465",
"RC01466",
"RC01467",
"RC01468",
"RC01469",
"RC01470",
"RC01471",
"RC01472",
"RC01473",
"RC01474",
"RC01475",
"RC01476",
"RC01477",
"RC01478",
"RC01479",
"RC03910",
"RC03921",
"RC04947"
]
},
{
"targ_id": "T019001",
"parent_targ_id": "T019",
"full_name": "c ring ",
"abbrev": "c ring ",
"protein_names": "",
"related_func_ids": "F020103",
"category": "enzyme structure",
"subcategories": "",
"Uniport_ID": "",
"Uniprot_name": "",
"EC_numbers": "",
"gene_symbol": "",
"gene_synonyms": "",
"gene_synonyms_links": "",
"gene_name": "",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "",
"omim_id": "",
"genecard_link": "",
"locs": "",
"reaction": "",
"targ_desciption": "",
"references": []
},
{
"targ_id": "T019002",
"parent_targ_id": "T019",
"full_name": "Fo subunits",
"abbrev": "Fo subunits",
"protein_names": "",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "",
"Uniprot_name": "",
"EC_numbers": "",
"gene_symbol": "",
"gene_synonyms": "",
"gene_synonyms_links": "",
"gene_name": "",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "",
"omim_id": "",
"genecard_link": "",
"locs": "",
"reaction": "",
"targ_desciption": "",
"references": [
"RC01182",
"RC01183",
"RC01184",
"RC01185",
"RC01186",
"RC01187",
"RC01188",
"RC01189",
"RC01190",
"RC01191",
"RC01192",
"RC01193",
"RC01194",
"RC01200",
"RC01201",
"RC01213",
"RC01214",
"RC01215",
"RC01216",
"RC01217",
"RC01218",
"RC01219",
"RC01220",
"RC01221",
"RC01222",
"RC01223",
"RC01225",
"RC01242"
]
},
{
"targ_id": "T019002001",
"parent_targ_id": "T019002",
"full_name": "ATP synthase F(0) complex subunit C1, mitochondrial",
"abbrev": "Fo subunit C1",
"protein_names": "ATP synthase F(0) complex subunit C1, mitochondrial (ATP synthase lipid-binding protein) (ATP synthase membrane subunit c locus 1) (ATP synthase proteolipid P1) (ATP synthase proton-transporting mitochondrial F(0) complex subunit C1) (ATPase protein 9) (ATPase subunit c)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "P05496",
"Uniprot_name": "AT5G1_HUMAN",
"EC_numbers": "",
"gene_symbol": "ATP5MC1",
"gene_synonyms": "ATP5G, ATP5G1",
"gene_synonyms_links": "",
"gene_name": "ATP synthase membrane subunit c locus 1",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000159199",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion membrane; Multi-pass membrane protein",
"reaction": "",
"targ_desciption": "Mitochondrial membrane ATP synthase (F(1)F(0) ATP synthase orComplex V) produces ATP from ADP in the presence of a proton gradientacross the membrane which is generated by electron transport complexesof the respiratory chain. F-type ATPases consist of two structuraldomains, F(1) - containing the extramembraneous catalytic core and F(0)- containing the membrane proton channel, linked together by a centralstalk and a peripheral stalk. During catalysis, ATP synthesis in thecatalytic domain of F(1) is coupled via a rotary mechanism of thecentral stalk subunits to proton translocation. Part of the complexF(0) domain. A homomeric c-ring of probably 10 subunits is part of thecomplex rotary element.",
"references": []
},
{
"targ_id": "T019003",
"parent_targ_id": "T019",
"full_name": "F1 subunits",
"abbrev": "F1 subunits",
"protein_names": "",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "",
"Uniprot_name": "",
"EC_numbers": "",
"gene_symbol": "",
"gene_synonyms": "",
"gene_synonyms_links": "",
"gene_name": "",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "",
"omim_id": "",
"genecard_link": "",
"locs": "",
"reaction": "",
"targ_desciption": "",
"references": [
"RC01202",
"RC01203",
"RC01204",
"RC01205",
"RC01206",
"RC01207",
"RC01208",
"RC01209",
"RC01210",
"RC01211",
"RC01224",
"RC01226",
"RC01237",
"RC01238",
"RC01239"
]
},
{
"targ_id": "T019003001",
"parent_targ_id": "T019003",
"full_name": "ATP synthase subunit gamma, mitochondrial",
"abbrev": "F-ATPase gamma subunit",
"protein_names": "ATP synthase subunit gamma, mitochondrial (ATP synthase F1 subunit gamma) (F-ATPase gamma subunit)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "Translocase",
"Uniport_ID": "P36542",
"Uniprot_name": "ATPG_HUMAN",
"EC_numbers": "",
"gene_symbol": "ATP5F1C",
"gene_synonyms": "ATP5C, ATP5C1, ATP5CL1",
"gene_synonyms_links": "",
"gene_name": "ATP synthase F1 subunit gamma",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000165629",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Peripheral membrane protein; Matrix side",
"reaction": "",
"targ_desciption": "Mitochondrial membrane ATP synthase (F(1)F(0) ATP synthase orComplex V) produces ATP from ADP in the presence of a proton gradientacross the membrane which is generated by electron transport complexesof the respiratory chain. F-type ATPases consist of two structuraldomains, F(1) - containing the extramembraneous catalytic core, andF(0) - containing the membrane proton channel, linked together by acentral stalk and a peripheral stalk. During catalysis, ATP synthesisin the catalytic domain of F(1) is coupled via a rotary mechanism ofthe central stalk subunits to proton translocation. Part of the complexF(1) domain and the central stalk which is part of the complex rotaryelement. The gamma subunit protrudes into the catalytic domain formedof alpha(3)beta(3). Rotation of the central stalk against thesurrounding alpha(3)beta(3) subunits leads to hydrolysis of ATP inthree separate catalytic sites on the beta subunits.{ECO:0000250|UniProtKB:P05631}.",
"references": []
},
{
"targ_id": "T019003002",
"parent_targ_id": "T019003",
"full_name": "ATP synthase subunit delta, mitochondrial",
"abbrev": "ATP synthase F1 subunit delta",
"protein_names": "ATP synthase subunit delta, mitochondrial (ATP synthase F1 subunit delta) (F-ATPase delta subunit)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "Translocase",
"Uniport_ID": "P30049",
"Uniprot_name": "ATPD_HUMAN",
"EC_numbers": "",
"gene_symbol": "ATP5F1D",
"gene_synonyms": "ATP5D",
"gene_synonyms_links": "",
"gene_name": "ATP synthase F1 subunit delta",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000099624",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion; Mitochondrion inner membrane",
"reaction": "",
"targ_desciption": "Mitochondrial membrane ATP synthase (F(1)F(0) ATP synthase orComplex V) produces ATP from ADP in the presence of a proton gradientacross the membrane which is generated by electron transport complexesof the respiratory chain (PubMed:29478781). F-type ATPases consist oftwo structural domains, F(1) - containing the extramembraneouscatalytic core, and F(0) - containing the membrane proton channel,linked together by a central stalk and a peripheral stalk. Duringcatalysis, ATP turnover in the catalytic domain of F(1) is coupled viaa rotary mechanism of the central stalk subunits to protontranslocation. Part of the complex F(1) domain and of the central stalkwhich is part of the complex rotary element. Rotation of the centralstalk against the surrounding alpha(3)beta(3) subunits leads tohydrolysis of ATP in three separate catalytic sites on the betasubunits (PubMed:1531933). {ECO:0000269|PubMed:29478781,ECO:0000303|PubMed:1531933}.",
"references": []
},
{
"targ_id": "T019003003",
"parent_targ_id": "T019003",
"full_name": "ATP synthase subunit alpha, mitochondrial",
"abbrev": "ATP synthase F1 subunit alpha",
"protein_names": "ATP synthase subunit alpha, mitochondrial (ATP synthase F1 subunit alpha)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "Translocase",
"Uniport_ID": "P25705",
"Uniprot_name": "ATPA_HUMAN",
"EC_numbers": "",
"gene_symbol": "ATP5F1A",
"gene_synonyms": "ATP5A, ATP5A1, ATP5AL2, ATPM, hATP1, OMR, ORM",
"gene_synonyms_links": "",
"gene_name": "ATP synthase F1 subunit alpha",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000152234",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion; Mitochondrion inner membrane; Peripheral membrane protein; Matrix side; Cell membrane; Peripheral membrane protein; Extracellular side",
"reaction": "",
"targ_desciption": "Mitochondrial membrane ATP synthase (F(1)F(0) ATP synthase orComplex V) produces ATP from ADP in the presence of a proton gradientacross the membrane which is generated by electron transport complexesof the respiratory chain. F-type ATPases consist of two structuraldomains, F(1) - containing the extramembraneous catalytic core, andF(0) - containing the membrane proton channel, linked together by acentral stalk and a peripheral stalk. During catalysis, ATP synthesisin the catalytic domain of F(1) is coupled via a rotary mechanism ofthe central stalk subunits to proton translocation. Subunits alpha andbeta form the catalytic core in F(1). Rotation of the central stalkagainst the surrounding alpha(3)beta(3) subunits leads to hydrolysis ofATP in three separate catalytic sites on the beta subunits. Subunitalpha does not bear the catalytic high-affinity ATP-binding sites (Bysimilarity). Binds the bacterial siderophore enterobactin and canpromote mitochondrial accumulation of enterobactin-derived iron ions(PubMed:30146159). {ECO:0000250|UniProtKB:P19483,ECO:0000269|PubMed:30146159}.",
"references": [
"RC01235"
]
},
{
"targ_id": "T019003004",
"parent_targ_id": "T019003",
"full_name": "ATP synthase subunit beta, mitochondrial",
"abbrev": "ATP synthase F1 subunit beta",
"protein_names": "ATP synthase subunit beta, mitochondrial (EC 7.1.2.2) (ATP synthase F1 subunit beta)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "Translocase",
"Uniport_ID": "P06576",
"Uniprot_name": "ATPB_HUMAN",
"EC_numbers": "7.1.2.2",
"gene_symbol": "ATP5F1B",
"gene_synonyms": "ATP5B, ATPSB",
"gene_synonyms_links": "",
"gene_name": "ATP synthase F1 subunit beta",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000110955",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Peripheral membrane protein; Matrix side",
"reaction": "ATP + 4 H(+)(in) + H2O = ADP + 5 H(+)(out) + phosphate",
"targ_desciption": "Mitochondrial membrane ATP synthase (F(1)F(0) ATP synthase orComplex V) produces ATP from ADP in the presence of a proton gradientacross the membrane which is generated by electron transport complexesof the respiratory chain. F-type ATPases consist of two structuraldomains, F(1) - containing the extramembraneous catalytic core, andF(0) - containing the membrane proton channel, linked together by acentral stalk and a peripheral stalk. During catalysis, ATP synthesisin the catalytic domain of F(1) is coupled via a rotary mechanism ofthe central stalk subunits to proton translocation. Subunits alpha andbeta form the catalytic core in F(1). Rotation of the central stalkagainst the surrounding alpha(3)beta(3) subunits leads to hydrolysis ofATP in three separate catalytic sites on the beta subunits.",
"references": [
"RC01236"
]
},
{
"targ_id": "T019004",
"parent_targ_id": "T019",
"full_name": "ATP synthase subunit O, mitochondrial",
"abbrev": "OSCP",
"protein_names": "ATP synthase subunit O, mitochondrial (ATP synthase peripheral stalk subunit OSCP) (Oligomycin sensitivity conferral protein) (OSCP)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "Translocase",
"Uniport_ID": "P48047",
"Uniprot_name": "ATPO_HUMAN",
"EC_numbers": "",
"gene_symbol": "ATP5PO",
"gene_synonyms": "ATP5O, ATPO, OSCP",
"gene_synonyms_links": "",
"gene_name": "ATP synthase peripheral stalk subunit OSCP",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000241837",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion; Mitochondrion inner membrane",
"reaction": "",
"targ_desciption": "Mitochondrial membrane ATP synthase (F(1)F(0) ATP synthase orComplex V) produces ATP from ADP in the presence of a proton gradientacross the membrane which is generated by electron transport complexesof the respiratory chain. F-type ATPases consist of two structuraldomains, F(1) - containing the extramembraneous catalytic core and F(0)- containing the membrane proton channel, linked together by a centralstalk and a peripheral stalk. During catalysis, ATP synthesis in thecatalytic domain of F(1) is coupled via a rotary mechanism of thecentral stalk subunits to proton translocation. Part of the complexF(0) domain and the peripheric stalk, which acts as a stator to holdthe catalytic alpha(3)beta(3) subcomplex and subunit a/ATP6 staticrelative to the rotary elements.",
"references": [
"RC01253"
]
},
{
"targ_id": "T019005",
"parent_targ_id": "T019",
"full_name": "ATP synthase subunit g, mitochondrial",
"abbrev": "ATPase subunit g",
"protein_names": "ATP synthase subunit g, mitochondrial (ATPase subunit g) (ATP synthase membrane subunit g)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "Translocase",
"Uniport_ID": "O75964",
"Uniprot_name": "ATP5L_HUMAN",
"EC_numbers": "",
"gene_symbol": "ATP5MG",
"gene_synonyms": "ATP5JG, ATP5L",
"gene_synonyms_links": "",
"gene_name": "ATP synthase membrane subunit g",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000167283",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion; Mitochondrion inner membrane",
"reaction": "",
"targ_desciption": "Mitochondrial membrane ATP synthase (F(1)F(0) ATP synthase orComplex V) produces ATP from ADP in the presence of a proton gradientacross the membrane which is generated by electron transport complexesof the respiratory chain. F-type ATPases consist of two structuraldomains, F(1) - containing the extramembraneous catalytic core, andF(0) - containing the membrane proton channel, linked together by acentral stalk and a peripheral stalk. During catalysis, ATP synthesisin the catalytic domain of F(1) is coupled via a rotary mechanism ofthe central stalk subunits to proton translocation. Part of the complexF(0) domain. Minor subunit located with subunit a in the membrane.",
"references": []
},
{
"targ_id": "T019006",
"parent_targ_id": "T019",
"full_name": "ATP synthase subunit e, mitochondrial",
"abbrev": "ATPase subunit e",
"protein_names": "ATP synthase subunit e, mitochondrial (ATPase subunit e) (ATP synthase membrane subunit e) [Cleaved into: ATP synthase subunit e, mitochondrial, N-terminally processed]",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "Translocase",
"Uniport_ID": "P56385",
"Uniprot_name": "ATP5I_HUMAN",
"EC_numbers": "",
"gene_symbol": "ATP5ME",
"gene_synonyms": "ATP5I",
"gene_synonyms_links": "",
"gene_name": "ATP synthase membrane subunit e",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000169020",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion; Mitochondrion inner membrane",
"reaction": "",
"targ_desciption": "Mitochondrial membrane ATP synthase (F(1)F(0) ATP synthase orComplex V) produces ATP from ADP in the presence of a proton gradientacross the membrane which is generated by electron transport complexesof the respiratory chain. F-type ATPases consist of two structuraldomains, F(1) - containing the extramembraneous catalytic core, andF(0) - containing the membrane proton channel, linked together by acentral stalk and a peripheral stalk. During catalysis, ATP synthesisin the catalytic domain of F(1) is coupled via a rotary mechanism ofthe central stalk subunits to proton translocation. Part of the complexF(0) domain. Minor subunit located with subunit a in the membrane.",
"references": []
},
{
"targ_id": "T019007",
"parent_targ_id": "T019",
"full_name": "ATP synthase subunit epsilon, mitochondrial",
"abbrev": "ATPase subunit epsilon",
"protein_names": "ATP synthase subunit epsilon, mitochondrial (ATPase subunit epsilon) (ATP synthase F1 subunit epsilon)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "Hydrolase",
"Uniport_ID": "P56381",
"Uniprot_name": "ATP5E_HUMAN",
"EC_numbers": "",
"gene_symbol": "ATP5F1E",
"gene_synonyms": "ATP5E",
"gene_synonyms_links": "",
"gene_name": "ATP synthase F1 subunit epsilon",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000124172",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion; Mitochondrion inner membrane",
"reaction": "",
"targ_desciption": "Mitochondrial membrane ATP synthase (F(1)F(0) ATP synthase orComplex V) produces ATP from ADP in the presence of a proton gradientacross the membrane which is generated by electron transport complexesof the respiratory chain. F-type ATPases consist of two structuraldomains, F(1) - containing the extramembraneous catalytic core, andF(0) - containing the membrane proton channel, linked together by acentral stalk and a peripheral stalk. During catalysis, ATP synthesisin the catalytic domain of F(1) is coupled via a rotary mechanism ofthe central stalk subunits to proton translocation. Part of the complexF(1) domain and of the central stalk which is part of the complexrotary element. Rotation of the central stalk against the surroundingalpha(3)beta(3) subunits leads to hydrolysis of ATP in three separatecatalytic sites on the beta subunits (By similarity). {ECO:0000250}.",
"references": []
},
{
"targ_id": "T019008",
"parent_targ_id": "T019",
"full_name": "ATP synthase mitochondrial F1 complex assembly factor 2",
"abbrev": "ATP12 homolog",
"protein_names": "ATP synthase mitochondrial F1 complex assembly factor 2 (ATP12 homolog)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "Chaperone",
"Uniport_ID": "Q8N5M1",
"Uniprot_name": "ATPF2_HUMAN",
"EC_numbers": "",
"gene_symbol": "ATPAF2",
"gene_synonyms": "ATP12, Atp12p, LP3663, MGC29736",
"gene_synonyms_links": "",
"gene_name": "ATP synthase mitochondrial F1 complex assembly factor 2",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000171953",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion",
"reaction": "",
"targ_desciption": "May play a role in the assembly of the F1 component of themitochondrial ATP synthase (ATPase). {ECO:0000269|PubMed:11410595}.",
"references": []
},
{
"targ_id": "T019009",
"parent_targ_id": "T019",
"full_name": "Transmembrane protein 70, mitochondrial",
"abbrev": "",
"protein_names": "Transmembrane protein 70, mitochondrial",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "Transporter",
"Uniport_ID": "Q9BUB7",
"Uniprot_name": "TMM70_HUMAN",
"EC_numbers": "",
"gene_symbol": "TMEM70",
"gene_synonyms": "FLJ20533",
"gene_synonyms_links": "",
"gene_name": "Transmembrane protein 70",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000175606",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Multi-pass membrane protein",
"reaction": "",
"targ_desciption": "Involved in biogenesis of mitochondrial ATP synthase.{ECO:0000269|PubMed:18953340, ECO:0000269|PubMed:20937241}.",
"references": []
},
{
"targ_id": "T019010",
"parent_targ_id": "T019",
"full_name": "ATP synthase membrane subunit DAPIT, mitochondrial",
"abbrev": "Diabetes-associated protein in insulin-sensitive tissues",
"protein_names": "ATP synthase membrane subunit DAPIT, mitochondrial (Diabetes-associated protein in insulin-sensitive tissues) (HCV F-transactivated protein 2) (Up-regulated during skeletal muscle growth protein 5)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "Q96IX5",
"Uniprot_name": "ATPMD_HUMAN",
"EC_numbers": "",
"gene_symbol": "ATP5MD",
"gene_synonyms": "bA792D24.4, DAPIT, MGC14697, USMG5",
"gene_synonyms_links": "",
"gene_name": "ATP synthase membrane subunit DAPIT",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000173915",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion membrane; Single-pass membrane protein",
"reaction": "",
"targ_desciption": "Mitochondrial membrane ATP synthase (F(1)F(0) ATP synthase orComplex V) produces ATP from ADP in the presence of a proton gradientacross the membrane which is generated by electron transport complexesof the respiratory chain. F-type ATPases consist of two structuraldomains, F(1) - containing the extramembraneous catalytic core and F(0)- containing the membrane proton channel, linked together by a centralstalk and a peripheral stalk. During catalysis, ATP synthesis in thecatalytic domain of F(1) is coupled via a rotary mechanism of thecentral stalk subunits to proton translocation (PubMed:29917077).ATP5MD is a minor subunit of the mitochondrial membrane ATP synthaserequired for dimerization of the ATP synthase complex and as suchregulates ATP synthesis in the mitochondria (PubMed:21345788,PubMed:29917077). {ECO:0000269|PubMed:21345788,ECO:0000269|PubMed:29917077, ECO:0000303|PubMed:29917077}.",
"references": []
},
{
"targ_id": "T019011",
"parent_targ_id": "T019",
"full_name": "ATP synthase subunit a",
"abbrev": "F-ATPase protein 6",
"protein_names": "ATP synthase subunit a (F-ATPase protein 6)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "Transporter",
"Uniport_ID": "P00846",
"Uniprot_name": "ATP6_HUMAN",
"EC_numbers": "",
"gene_symbol": "MT-ATP6",
"gene_synonyms": "ATP6, ATPase-6, MTATP6, RP, Su6m",
"gene_synonyms_links": "",
"gene_name": "Mitochondrially encoded ATP synthase membrane subunit 6",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000198899",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Multi-pass membrane protein",
"reaction": "",
"targ_desciption": "Mitochondrial membrane ATP synthase (F(1)F(0) ATP synthase orComplex V) produces ATP from ADP in the presence of a proton gradientacross the membrane which is generated by electron transport complexesof the respiratory chain. F-type ATPases consist of two structuraldomains, F(1) - containing the extramembraneous catalytic core and F(0)- containing the membrane proton channel, linked together by a centralstalk and a peripheral stalk. During catalysis, ATP synthesis in thecatalytic domain of F(1) is coupled via a rotary mechanism of thecentral stalk subunits to proton translocation. Key component of theproton channel. it may play a direct role in the translocation ofprotons across the membrane.",
"references": [
"RC01195",
"RC01196",
"RC01197",
"RC01198",
"RC01199"
]
},
{
"targ_id": "T019012",
"parent_targ_id": "T019",
"full_name": "ATP synthase protein 8",
"abbrev": "A6L",
"protein_names": "ATP synthase protein 8 (A6L) (F-ATPase subunit 8)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "Transporter",
"Uniport_ID": "P03928",
"Uniprot_name": "ATP8_HUMAN",
"EC_numbers": "",
"gene_symbol": "MT-ATP8",
"gene_synonyms": "A6L, ATP8, MTATP8",
"gene_synonyms_links": "",
"gene_name": "Mitochondrially encoded ATP synthase membrane subunit 8",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000228253",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion membrane; Single-pass membrane protein",
"reaction": "",
"targ_desciption": "Mitochondrial membrane ATP synthase (F(1)F(0) ATP synthase orComplex V) produces ATP from ADP in the presence of a proton gradientacross the membrane which is generated by electron transport complexesof the respiratory chain. F-type ATPases consist of two structuraldomains, F(1) - containing the extramembraneous catalytic core and F(0)- containing the membrane proton channel, linked together by a centralstalk and a peripheral stalk. During catalysis, ATP synthesis in thecatalytic domain of F(1) is coupled via a rotary mechanism of thecentral stalk subunits to proton translocation. Part of the complexF(0) domain. Minor subunit located with subunit a in the membrane (Bysimilarity). {ECO:0000250}.",
"references": []
},
{
"targ_id": "T019013",
"parent_targ_id": "T019",
"full_name": "V-type proton ATPase catalytic subunit A",
"abbrev": "V-ATPase subunit A",
"protein_names": "V-ATPase 69 kDa subunit; Vacuolar ATPase isoform VA68; Vacuolar proton pump subunit alpha",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "P38606",
"Uniprot_name": "VATA_HUMAN",
"EC_numbers": "7.1.2.2",
"gene_symbol": "ATP6A1 ATP6V1A1 VPP2",
"gene_synonyms": "",
"gene_synonyms_links": "",
"gene_name": "",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "",
"omim_id": "",
"genecard_link": "",
"locs": "Cytoplasm",
"reaction": "ATP + 4 H(+)(in) + H2O = ADP + 5 H(+)(out) + phosphate",
"targ_desciption": "Catalytic subunit of the peripheral V1 complex of vacuolar ATPase. V-ATPase vacuolar ATPase is responsible for acidifying a variety of intracellular compartments in eukaryotic cells. In aerobic conditions, involved in intracellular iron homeostasis, thus triggering the activity of Fe2+ prolyl hydroxylase (PHD) enzymes, and leading to HIF1A hydroxylation and subsequent proteasomal degradation (PubMed:28296633). May play a role in neurite development and synaptic connectivity (PubMed:29668857)",
"references": []
},
{
"targ_id": "T020",
"parent_targ_id": "T",
"full_name": "ATP",
"abbrev": "ATP",
"protein_names": "",
"related_func_ids": "F020103",
"category": "molecule",
"subcategories": "",
"Uniport_ID": "",
"Uniprot_name": "",
"EC_numbers": "",
"gene_symbol": "",
"gene_synonyms": "",
"gene_synonyms_links": "",
"gene_name": "",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "",
"omim_id": "",
"genecard_link": "",
"locs": "",
"reaction": "",
"targ_desciption": "",
"references": [
"RC00492",
"RC00496",
"RC00758",
"RC00759",
"RC00760",
"RC00761",
"RC00762",
"RC00763",
"RC00764",
"RC00765",
"RC00766",
"RC00767",
"RC00768",
"RC00769",
"RC00770",
"RC00771",
"RC00772"
]
},
{
"targ_id": "T021",
"parent_targ_id": "T",
"full_name": "carnitine palmitoyltransferases I",
"abbrev": "CPT-I",
"protein_names": "",
"related_func_ids": "F02060101",
"category": "enzyme",
"subcategories": "",
"Uniport_ID": "",
"Uniprot_name": "",
"EC_numbers": "2.3.1.21",
"gene_symbol": "",
"gene_synonyms": "",
"gene_synonyms_links": "",
"gene_name": "",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "",
"omim_id": "",
"genecard_link": "",
"locs": "",
"reaction": "(R)-carnitine + hexadecanoyl-CoA = CoA + O-hexadecanoyl-(R)-carnitine",
"targ_desciption": "",
"references": [
"RC00865",
"RC00872",
"RC00945",
"RC00966",
"RC03532",
"RC03533",
"RC03534",
"RC03535",
"RC03547",
"RC03548",
"RC03549"
]
},
{
"targ_id": "T021001",
"parent_targ_id": "T021",
"full_name": "Carnitine O-palmitoyltransferase 1, liver isoform, CPT1-L, EC 2.3.1.21",
"abbrev": "CPT-1A",
"protein_names": "Carnitine O-palmitoyltransferase 1, liver isoform (CPT1-L) (EC 2.3.1.21) (Carnitine O-palmitoyltransferase I, liver isoform) (CPT I) (CPTI-L) (Carnitine palmitoyltransferase 1A)",
"related_func_ids": "F02060101",
"category": "protein subunit",
"subcategories": "Acyltransferase, Transferase",
"Uniport_ID": "P50416",
"Uniprot_name": "CPT1A_HUMAN",
"EC_numbers": "2.3.1.21",
"gene_symbol": "CPT1A",
"gene_synonyms": "CPT1, CPT1-L, L-CPT1",
"gene_synonyms_links": "",
"gene_name": "Carnitine palmitoyltransferase 1A",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000110090",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion outer membrane; Multi-pass membrane protein",
"reaction": "(R)-carnitine + hexadecanoyl-CoA = CoA + O-hexadecanoyl-(R)-carnitine",
"targ_desciption": "Catalyzes the transfer of the acyl group of long-chain fattyacid-CoA conjugates onto carnitine, an essential step for themitochondrial uptake of long-chain fatty acids and their subsequentbeta-oxidation in the mitochondrion. Plays an important role intriglyceride metabolism.",
"references": [
"RC03673",
"RC05000",
"RC05001",
"RC05002",
"RC05003",
"RC05004",
"RC05005",
"RC05006",
"RC05007",
"RC05008",
"RC05009"
]
},
{
"targ_id": "T021002",
"parent_targ_id": "T021",
"full_name": "Carnitine O-palmitoyltransferase 1, muscle isoform",
"abbrev": "CPT-1B",
"protein_names": "Carnitine O-palmitoyltransferase 1, muscle isoform (CPT1-M) (EC 2.3.1.21) (Carnitine O-palmitoyltransferase I, muscle isoform) (CPT I) (CPTI-M) (Carnitine palmitoyltransferase 1B) (Carnitine palmitoyltransferase I-like protein)",
"related_func_ids": "F02060101",
"category": "protein subunit",
"subcategories": "Acyltransferase, Transferase",
"Uniport_ID": "Q92523",
"Uniprot_name": "CPT1B_HUMAN",
"EC_numbers": "2.3.1.21",
"gene_symbol": "CPT1B",
"gene_synonyms": "CPT1-M, M-CPT1",
"gene_synonyms_links": "",
"gene_name": "Carnitine palmitoyltransferase 1B",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000205560",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion outer membrane; Multi-pass membrane protein",
"reaction": "(R)-carnitine + hexadecanoyl-CoA = CoA + O-hexadecanoyl-(R)-carnitine",
"targ_desciption": "",
"references": [
"RC05010",
"RC05011",
"RC05012",
"RC05013",
"RC05014",
"RC05015",
"RC05016",
"RC05017",
"RC05018",
"RC05019"
]
},
{
"targ_id": "T021003",
"parent_targ_id": "T021",
"full_name": "Carnitine O-palmitoyltransferase 1, brain isoform, CPT1-B, EC 2.3.1.21",
"abbrev": "CPT-1C",
"protein_names": "Carnitine O-palmitoyltransferase 1, brain isoform (CPT1-B) (EC 2.3.1.21) (CPT IC) (Carnitine O-palmitoyltransferase I, brain isoform) (CPTI-B) (Carnitine palmitoyltransferase 1C)",
"related_func_ids": "F02060101",
"category": "protein subunit",
"subcategories": "Acyltransferase, Transferase",
"Uniport_ID": "Q8TCG5",
"Uniprot_name": "CPT1C_HUMAN",
"EC_numbers": "2.3.1.21",
"gene_symbol": "CPT1C",
"gene_synonyms": "CPT1P, CPTIC, FLJ23809",
"gene_synonyms_links": "",
"gene_name": "Carnitine palmitoyltransferase 1C",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000169169",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion outer membrane; Multi-pass membrane protein; Cell junction, synapse; Cell projection, dendrite; Cell projection, axon; Endoplasmic reticulum",
"reaction": "(R)-carnitine + hexadecanoyl-CoA = CoA + O-hexadecanoyl-(R)-carnitine",
"targ_desciption": "May play a role in lipid metabolic process.{ECO:0000269|PubMed:25751282}.",
"references": []
},
{
"targ_id": "T022",
"parent_targ_id": "T",
"full_name": "Carnitine O-palmitoyltransferase 2, mitochondrial, EC 2.3.1.21",
"abbrev": "CPT-II",
"protein_names": "Carnitine O-palmitoyltransferase 2, mitochondrial (EC 2.3.1.21) (Carnitine palmitoyltransferase II) (CPT II)",
"related_func_ids": "F02060101",
"category": "enzyme",
"subcategories": "Acyltransferase, Transferase",
"Uniport_ID": "P23786",
"Uniprot_name": "CPT2_HUMAN",
"EC_numbers": "2.3.1.21",
"gene_symbol": "CPT2",
"gene_synonyms": "CPT1, CPTASE",
"gene_synonyms_links": "",
"gene_name": "Carnitine palmitoyltransferase 2",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000157184",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Peripheral membrane protein; Matrix side",
"reaction": "(R)-carnitine + hexadecanoyl-CoA = CoA + O-hexadecanoyl-(R)-carnitine",
"targ_desciption": "",
"references": [
"RC00866",
"RC00873",
"RC05020",
"RC05021",
"RC05022",
"RC05023",
"RC05024",
"RC05025",
"RC05026",
"RC05027",
"RC05028",
"RC05029"
]
},
{
"targ_id": "T023",
"parent_targ_id": "T",
"full_name": "Mitochondrial carnitine/acylcarnitine carrier protein",
"abbrev": "CACT",
"protein_names": "Mitochondrial carnitine/acylcarnitine carrier protein (Carnitine/acylcarnitine translocase) (CAC) (Solute carrier family 25 member 20)",
"related_func_ids": "F02060101; F02110107",
"category": "protein",
"subcategories": "Transporter",
"Uniport_ID": "O43772",
"Uniprot_name": "MCAT_HUMAN",
"EC_numbers": "",
"gene_symbol": "SLC25A20",
"gene_synonyms": "CAC, CACT",
"gene_synonyms_links": "",
"gene_name": "Solute carrier family 25 member 20",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000178537",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Multi-pass membrane protein",
"reaction": "",
"targ_desciption": "Mediates the transport of acylcarnitines of different length across the mitochondrial inner membrane from the cytosol to themitochondrial matrix for their oxidation by the mitochondrial fatty acid-oxidation pathway.",
"references": []
},
{
"targ_id": "T024",
"parent_targ_id": "T",
"full_name": "Enoyl-CoA hydratase, mitochondrial, EC 4.2.1.17",
"abbrev": "ECHS1",
"protein_names": "Enoyl-CoA hydratase, mitochondrial (EC 4.2.1.17) (Enoyl-CoA hydratase 1) (Short-chain enoyl-CoA hydratase) (SCEH)",
"related_func_ids": "F02060101",
"category": "enzyme",
"subcategories": "Lyase",
"Uniport_ID": "P30084",
"Uniprot_name": "ECHM_HUMAN",
"EC_numbers": "4.2.1.17",
"gene_symbol": "ECHS1",
"gene_synonyms": "SCEH",
"gene_synonyms_links": "",
"gene_name": "Enoyl-CoA hydratase, short chain 1",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000127884",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion matrix",
"reaction": "a (3S)-hydroxyacyl-CoA = a (2E)-enoyl-CoA + H2O; a 4-saturated-(3S)-hydroxyacyl-CoA = a (3E)-enoyl-CoA + H2O; (3S)-hydroxybutanoyl-CoA = (2E)-butenoyl-CoA + H2O; 3-hydroxyisovaleryl-CoA = 3-methylbut-2-enoyl-CoA + H2O; 3-hydroxypropanoyl-CoA = acryloyl-CoA + H2O; 3-hydroxybutanoyl-CoA = (2E)-butenoyl-CoA + H2O",
"targ_desciption": "Straight-chain enoyl-CoA thioesters from C4 up to at least C16 are processed, although with decreasing catalytic rate. Has high substrate specificity for crotonyl-CoA and moderate specificity for acryloyl-CoA, 3-methylcrotonyl-CoA and methacrylyl-CoA. It is noteworthy that binds tiglyl-CoA, but hydrates only a small amount of this substrate. {ECO:0000269|PubMed:26251176}.",
"references": []
},
{
"targ_id": "T025",
"parent_targ_id": "T",
"full_name": "Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial, HCDH, EC 1.1.1.35",
"abbrev": "HCDH",
"protein_names": "Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial (HCDH) (EC 1.1.1.35) (Medium and short-chain L-3-hydroxyacyl-coenzyme A dehydrogenase) (Short-chain 3-hydroxyacyl-CoA dehydrogenase)",
"related_func_ids": "F02060101",
"category": "enzyme",
"subcategories": "Oxidoreductase",
"Uniport_ID": "Q16836",
"Uniprot_name": "HCDH_HUMAN",
"EC_numbers": "1.1.1.35",
"gene_symbol": "HADH",
"gene_synonyms": "HADH1, HADHSC, SCHAD",
"gene_synonyms_links": "",
"gene_name": "Hydroxyacyl-CoA dehydrogenase",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000138796",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion matrix",
"reaction": "a (3S)-hydroxyacyl-CoA + NAD(+) = a 3-oxoacyl-CoA + H(+) +NADH",
"targ_desciption": "Plays an essential role in the mitochondrial beta-oxidationof short chain fatty acids. Exerts it highest activity toward 3-hydroxybutyryl-CoA.",
"references": []
},
{
"targ_id": "T026",
"parent_targ_id": "T",
"full_name": "3-ketoacyl-CoA thiolase, mitochondrial",
"abbrev": "Beta-ketothiolase",
"protein_names": "3-ketoacyl-CoA thiolase, mitochondrial (EC 2.3.1.16) (Acetyl-CoA acetyltransferase) (EC 2.3.1.9) (Acetyl-CoA acyltransferase) (Acyl-CoA hydrolase, mitochondrial) (EC 3.1.2.-) (EC 3.1.2.1) (EC 3.1.2.2) (Beta-ketothiolase) (Mitochondrial 3-oxoacyl-CoA thiolase) (T1)",
"related_func_ids": "F02060101; F0805",
"category": "enzyme",
"subcategories": "Acyltransferase, Hydrolase, Transferase",
"Uniport_ID": "P42765",
"Uniprot_name": "THIM_HUMAN",
"EC_numbers": "2.3.1.16; 2.3.1.9; 3.1.2.-; 3.1.2.1; 3.1.2.2",
"gene_symbol": "ACAA2",
"gene_synonyms": "DSAEC",
"gene_synonyms_links": "",
"gene_name": "Acetyl-CoA acyltransferase 2",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000167315",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion",
"reaction": "acetyl-CoA + an acyl-CoA = a 3-oxoacyl-CoA + CoA; 2 acetyl-CoA = acetoacetyl-CoA + CoA; acetyl-CoA + H2O = acetate + CoA + H(+); H2O + propanoyl-CoA = CoA + H(+) + propanoate; butanoyl-CoA + H2O = butanoate + CoA + H(+); H2O + hexanoyl-CoA = CoA + H(+) + hexanoate; H2O + octanoyl-CoA = CoA + H(+) + octanoate; decanoyl-CoA + H2O = CoA + decanoate + H(+); dodecanoyl-CoA + H2O = CoA + dodecanoate + H(+); H2O + tetradecanoyl-CoA = CoA + H(+) + tetradecanoate; H2O + hexadecanoyl-CoA = CoA + H(+) + hexadecanoate",
"targ_desciption": "In the production of energy from fats, this is one of the enzymes that catalyzes the last step of the mitochondrial beta-oxidation pathway, an aerobic process breaking down fatty acids intoacetyl-CoA (Probable). Using free coenzyme A/CoA, catalyzes thethiolytic cleavage of medium- to long-chain unbranched 3-oxoacyl-CoAs into acetyl-CoA and a fatty acyl-CoA shortened by two carbon atoms(Probable). Also catalyzes the condensation of two acetyl-CoA moleculesinto acetoacetyl-CoA and could be involved in the production of ketone bodies (Probable). Also displays hydrolase activity on various fatty acyl-CoAs (PubMed:25478839). Thereby, could be responsible for theproduction of acetate in a side reaction to beta-oxidation (Probable). Abolishes BNIP3-mediated apoptosis and mitochondrial damage(PubMed:18371312). {ECO:0000269|PubMed:18371312,ECO:0000269|PubMed:25478839, ECO:0000305|PubMed:25478839}.",
"references": [
"RC00388",
"RC00389",
"RC00390",
"RC00391"
]
},
{
"targ_id": "T027",
"parent_targ_id": "T",
"full_name": "Long-chain-fatty-acid--CoA ligase",
"abbrev": "ACSL",
"protein_names": "",
"related_func_ids": "F02060101",
"category": "enzyme family",
"subcategories": "",
"Uniport_ID": "",
"Uniprot_name": "",
"EC_numbers": "6.2.1.3",
"gene_symbol": "",
"gene_synonyms": "",
"gene_synonyms_links": "",
"gene_name": "",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "",
"omim_id": "",
"genecard_link": "",
"locs": "",
"reaction": "",
"targ_desciption": "",
"references": [
"RC03531"
]
},
{
"targ_id": "T027001",
"parent_targ_id": "T027",
"full_name": "Long-chain-fatty-acid--CoA ligase 1",
"abbrev": "ACSL1",
"protein_names": "Long-chain-fatty-acid--CoA ligase 1 (EC 6.2.1.3) (Acyl-CoA synthetase 1) (ACS1) (Arachidonate--CoA ligase) (EC 6.2.1.15) (Long-chain acyl-CoA synthetase 1) (LACS 1) (Long-chain acyl-CoA synthetase 2) (LACS 2) (Long-chain fatty acid-CoA ligase 2) (Palmitoyl-CoA ligase 1) (Palmitoyl-CoA ligase 2) (Phytanate--CoA ligase) (EC 6.2.1.24)",
"related_func_ids": "F02060101",
"category": "enzyme",
"subcategories": "Ligase",
"Uniport_ID": "P33121",
"Uniprot_name": "ACSL1_HUMAN",
"EC_numbers": "6.2.1.3; 6.2.1.15; 6.2.1.24",
"gene_symbol": "ACSL1",
"gene_synonyms": "ACS1, FACL1, FACL2, LACS, LACS1, LACS2",
"gene_synonyms_links": "",
"gene_name": "Acyl-CoA synthetase long chain family member 1",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000151726",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion outer membrane; Single-pass type III membrane protein; Peroxisome membrane; Single-pass type III membrane protein; Microsome membrane; Single-pass type III membrane protein; Endoplasmic reticulum membrane; Single-pass type III membrane protein",
"reaction": "a long-chain fatty acid + ATP + CoA = a long-chain fatty acyl-CoA + AMP + diphosphate; (5Z,8Z,11Z,14Z)-eicosatetraenoate + ATP + CoA =(5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoA + AMP + diphosphate; 3,7,11,15-tetramethylhexadecanoate + ATP + CoA = AMP +diphosphate + phytanoyl-CoA; ATP + CoA + hexadecanoate = AMP + diphosphate + hexadecanoyl-CoA; (E)-hexadec-2-enoate + ATP + CoA = (2E)-hexadecenoyl-CoA + AMP+ diphosphate; 2,6,10,14-tetramethylpentadecanoate + ATP + CoA = AMP +diphosphate + pristanoyl-CoA; 14,15-epoxy-(5Z,8Z,11Z)-eicosatrienoate + ATP + CoA = 14,15-epoxy-(5Z,8Z,11Z)-eicosatrienoyl-CoA + AMP + diphosphate; 5-hydroxy-(6E,8Z,11Z,14Z)-eicosatetraenoate + ATP + CoA = 5-hydroxy-(6E,8Z,11Z,14Z)-eicosatetraenoyl-CoA + AMP + diphosphate; 12-hydroxy-(5Z,8Z,10E,14Z)-eicosatetraenoate + ATP + CoA = 12-hydroxy-(5Z,8Z,10E,14Z)-eicosatetraenoyl-CoA + AMP + diphosphate; 15-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoate + ATP + CoA = 15-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoyl-CoA + AMP + diphosphate",
"targ_desciption": "Catalyzes the conversion of long-chain fatty acids to theiractive form acyl-CoAs for both synthesis of cellular lipids, anddegradation via beta-oxidation (PubMed:24269233, PubMed:22633490).Preferentially uses palmitoleate, oleate and linoleate(PubMed:24269233). Preferentially activates arachidonate thanepoxyeicosatrienoic acids (EETs) or hydroxyeicosatrienoic acids (HETEs)(By similarity). {ECO:0000250|UniProtKB:P18163,ECO:0000269|PubMed:22633490, ECO:0000269|PubMed:24269233}.",
"references": [
"RC00861",
"RC00862"
]
},
{
"targ_id": "T027002",
"parent_targ_id": "T027",
"full_name": "Long-chain-fatty-acid--CoA ligase 3",
"abbrev": "ACSL3",
"protein_names": "Long-chain-fatty-acid--CoA ligase 3 (EC 6.2.1.3) (Arachidonate--CoA ligase) (EC 6.2.1.15) (Long-chain acyl-CoA synthetase 3) (LACS 3)",
"related_func_ids": "F02060101",
"category": "enzyme",
"subcategories": "Ligase",
"Uniport_ID": "O95573",
"Uniprot_name": "ACSL3_HUMAN",
"EC_numbers": "6.2.1.3; 6.2.1.15",
"gene_symbol": "ACSL3",
"gene_synonyms": "ACS3, FACL3, PRO2194",
"gene_synonyms_links": "",
"gene_name": "Acyl-CoA synthetase long chain family member 3",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000123983",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion outer membrane; Single-pass type III membrane protein; Peroxisome membrane; Single-pass type III membrane protein; Microsome membrane; Single-pass type III membrane protein; Endoplasmic reticulum membrane; Single-pass type III membrane protein",
"reaction": "a long-chain fatty acid + ATP + CoA = a long-chain fatty acyl-CoA + AMP + diphosphate; (5Z,8Z,11Z,14Z)-eicosatetraenoate + ATP + CoA =(5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoA + AMP + diphosphate; (E)-hexadec-2-enoate + ATP + CoA = (2E)-hexadecenoyl-CoA + AMP+ diphosphate; 15-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoate + ATP + CoA = 15-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoyl-CoA + AMP + diphosphate; 12-hydroxy-(5Z,8Z,10E,14Z)-eicosatetraenoate + ATP + CoA = 12-hydroxy-(5Z,8Z,10E,14Z)-eicosatetraenoyl-CoA + AMP + diphosphate; 5-hydroxy-(6E,8Z,11Z,14Z)-eicosatetraenoate + ATP + CoA = 5-hydroxy-(6E,8Z,11Z,14Z)-eicosatetraenoyl-CoA + AMP + diphosphate; 14,15-epoxy-(5Z,8Z,11Z)-eicosatrienoate + ATP + CoA = 14,15-epoxy-(5Z,8Z,11Z)-eicosatrienoyl-CoA + AMP + diphosphate; 11,12-epoxy-(5Z,8Z,14Z)-eicosatrienoate + ATP + CoA = 11,12-epoxy-(5Z,8Z,14Z)-eicosatrienoyl-CoA + AMP + diphosphate",
"targ_desciption": "Acyl-CoA synthetases (ACSL) activates long-chain fatty acidsfor both synthesis of cellular lipids, and degradation via beta-oxidation (PubMed:22633490). Required for the incorporation of fattyacids into phosphatidylcholine, the major phospholipid located on thesurface of VLDL (very low density lipoproteins) (PubMed:18003621). Hasmainly an anabolic role in energy metabolism. Mediates hepaticlipogenesis. Preferentially uses myristate, laurate, arachidonate andeicosapentaenoate as substrates. Both isoforms exhibit the same levelof activity (By similarity). {ECO:0000250|UniProtKB:Q63151,ECO:0000269|PubMed:18003621, ECO:0000269|PubMed:22633490}.",
"references": []
},
{
"targ_id": "T027003",
"parent_targ_id": "T027",
"full_name": "Long-chain-fatty-acid--CoA ligase 4",
"abbrev": "ACSL4",
"protein_names": "Long-chain-fatty-acid--CoA ligase 4 (EC 6.2.1.3) (Arachidonate--CoA ligase) (EC 6.2.1.15) (Long-chain acyl-CoA synthetase 4) (LACS 4)",
"related_func_ids": "F02060101",
"category": "enzyme",
"subcategories": "Ligase",
"Uniport_ID": "O60488",
"Uniprot_name": "ACSL4_HUMAN",
"EC_numbers": "6.2.1.3; 6.2.1.15",
"gene_symbol": "ACSL4",
"gene_synonyms": "ACS4, FACL4, LACS4, MRX63, MRX68",
"gene_synonyms_links": "",
"gene_name": "Acyl-CoA synthetase long chain family member 4",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000068366",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion outer membrane; Single-pass type III membrane protein; Peroxisome membrane; Single-pass type III membrane protein; Microsome membrane; Single-pass type III membrane protein; Endoplasmic reticulum membrane; Single-pass type III membrane protein; Cell membrane",
"reaction": "a long-chain fatty acid + ATP + CoA = a long-chain fatty acyl-CoA + AMP + diphosphate; (5Z,8Z,11Z,14Z)-eicosatetraenoate + ATP + CoA =(5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoA + AMP + diphosphate; ATP + CoA + hexadecanoate = AMP + diphosphate + hexadecanoyl-CoA; (E)-hexadec-2-enoate + ATP + CoA = (2E)-hexadecenoyl-CoA + AMP+ diphosphate; 15-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoate + ATP + CoA = 15-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoyl-CoA + AMP + diphosphate; 12-hydroxy-(5Z,8Z,10E,14Z)-eicosatetraenoate + ATP + CoA = 12-hydroxy-(5Z,8Z,10E,14Z)-eicosatetraenoyl-CoA + AMP + diphosphate; 5-hydroxy-(6E,8Z,11Z,14Z)-eicosatetraenoate + ATP + CoA = 5-hydroxy-(6E,8Z,11Z,14Z)-eicosatetraenoyl-CoA + AMP + diphosphate; 5,6-epoxy-(8Z,11Z,14Z)-eicosatrienoate + ATP + CoA = 5,6-epoxy-(8Z,11Z,14Z)-eicosatrienoyl-CoA + AMP + diphosphate; 14,15-epoxy-(5Z,8Z,11Z)-eicosatrienoate + ATP + CoA = 14,15-epoxy-(5Z,8Z,11Z)-eicosatrienoyl-CoA + AMP + diphosphate; 11,12-epoxy-(5Z,8Z,14Z)-eicosatrienoate + ATP + CoA = 11,12-epoxy-(5Z,8Z,14Z)-eicosatrienoyl-CoA + AMP + diphosphate; 8,9-epoxy-(5Z,11Z,14Z)-eicosatrienoate + ATP + CoA = 8,9-epoxy-(5Z,11Z,14Z)-eicosatrienoyl-CoA + AMP + diphosphate",
"targ_desciption": "Catalyzes the conversion of long-chain fatty acids to theiractive form acyl-CoA for both synthesis of cellular lipids, anddegradation via beta-oxidation (PubMed:24269233, PubMed:22633490,PubMed:21242590). Preferentially activates arachidonate andeicosapentaenoate as substrates (PubMed:21242590). Preferentiallyactivates 8,9-EET > 14,15-EET > 5,6-EET > 11,12-EET. Modulates glucose-stimulated insulin secretion by regulating the levels of unesterifiedEETs (By similarity). Modulates prostaglandin E2 secretion(PubMed:21242590). {ECO:0000250|UniProtKB:O35547,ECO:0000269|PubMed:21242590, ECO:0000269|PubMed:22633490,ECO:0000269|PubMed:24269233}.",
"references": []
},
{
"targ_id": "T027004",
"parent_targ_id": "T027",
"full_name": "Long-chain-fatty-acid--CoA ligase 5",
"abbrev": "ACSL5",
"protein_names": "Long-chain-fatty-acid--CoA ligase 5 (EC 6.2.1.3) (Arachidonate--CoA ligase) (EC 6.2.1.15) (Long-chain acyl-CoA synthetase 5) (LACS 5)",
"related_func_ids": "F02060101",
"category": "enzyme",
"subcategories": "Ligase",
"Uniport_ID": "Q9ULC5",
"Uniprot_name": "ACSL5_HUMAN",
"EC_numbers": "6.2.1.3; 6.2.1.15",
"gene_symbol": "ACSL5",
"gene_synonyms": "ACS2, ACS5, FACL5",
"gene_synonyms_links": "",
"gene_name": "Acyl-CoA synthetase long chain family member 5",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000197142",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion; Endoplasmic reticulum; Mitochondrion outer membrane; Single-pass type III membrane protein; Endoplasmic reticulum membrane; Single-pass type III membrane protein; Cell membrane",
"reaction": "a long-chain fatty acid + ATP + CoA = a long-chain fatty acyl-CoA + AMP + diphosphate; (5Z,8Z,11Z,14Z)-eicosatetraenoate + ATP + CoA =(5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoA + AMP + diphosphate; ATP + CoA + hexadecanoate = AMP + diphosphate + hexadecanoyl-CoA; (E)-hexadec-2-enoate + ATP + CoA = (2E)-hexadecenoyl-CoA + AMP+ diphosphate; 15-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoate + ATP + CoA = 15-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoyl-CoA + AMP + diphosphate; 12-hydroxy-(5Z,8Z,10E,14Z)-eicosatetraenoate + ATP + CoA = 12-hydroxy-(5Z,8Z,10E,14Z)-eicosatetraenoyl-CoA + AMP + diphosphate; 5-hydroxy-(6E,8Z,11Z,14Z)-eicosatetraenoate + ATP + CoA = 5-hydroxy-(6E,8Z,11Z,14Z)-eicosatetraenoyl-CoA + AMP + diphosphate; 14,15-epoxy-(5Z,8Z,11Z)-eicosatrienoate + ATP + CoA = 14,15-epoxy-(5Z,8Z,11Z)-eicosatrienoyl-CoA + AMP + diphosphate; 11,12-epoxy-(5Z,8Z,14Z)-eicosatrienoate + ATP + CoA = 11,12-epoxy-(5Z,8Z,14Z)-eicosatrienoyl-CoA + AMP + diphosphate",
"targ_desciption": "Catalyzes the conversion of long-chain fatty acids to theiractive form acyl-CoAs for both synthesis of cellular lipids, anddegradation via beta-oxidation (PubMed:17681178, PubMed:24269233,PubMed:22633490). ACSL5 may activate fatty acids from exogenous sourcesfor the synthesis of triacylglycerol destined for intracellular storage(By similarity). Utilizes a wide range of saturated fatty acids with apreference for C16-C18 unsaturated fatty acids (By similarity). It wassuggested that it may also stimulate fatty acid oxidation (Bysimilarity). At the villus tip of the crypt-villus axis of the smallintestine may sensitize epithelial cells to apoptosis specificallytriggered by the death ligand TRAIL. May have a role in the survival ofglioma cells. {ECO:0000250, ECO:0000269|PubMed:17681178,ECO:0000269|PubMed:18806831, ECO:0000269|PubMed:19459852,ECO:0000269|PubMed:22633490, ECO:0000269|PubMed:24269233}.",
"references": []
},
{
"targ_id": "T027005",
"parent_targ_id": "T027",
"full_name": "Long-chain-fatty-acid--CoA ligase 6",
"abbrev": "ACSL6",
"protein_names": "Long-chain-fatty-acid--CoA ligase 6 (EC 6.2.1.3) (Arachidonate--CoA ligase) (EC 6.2.1.15) (Long-chain acyl-CoA synthetase 6) (LACS 6)",
"related_func_ids": "F02060101",
"category": "enzyme",
"subcategories": "Ligase",
"Uniport_ID": "Q9UKU0",
"Uniprot_name": "ACSL6_HUMAN",
"EC_numbers": "6.2.1.3; 6.2.1.15",
"gene_symbol": "ACSL6",
"gene_synonyms": "ACS2, FACL6, KIAA0837, LACS2, LACS5",
"gene_synonyms_links": "",
"gene_name": "Acyl-CoA synthetase long chain family member 6",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000164398",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion outer membrane; Single-pass type III membrane protein; Peroxisome membrane; Single-pass type III membrane protein; Microsome membrane; Single-pass type III membrane protein; Endoplasmic reticulum membrane; Single-pass type III membrane protein",
"reaction": "a long-chain fatty acid + ATP + CoA = a long-chain fatty acyl-CoA + AMP + diphosphate; (5Z,8Z,11Z,14Z)-eicosatetraenoate + ATP + CoA =(5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoA + AMP + diphosphate; ATP + CoA + hexadecanoate = AMP + diphosphate + hexadecanoyl-CoA; (E)-hexadec-2-enoate + ATP + CoA = (2E)-hexadecenoyl-CoA + AMP+ diphosphate; 15-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoate + ATP + CoA = 15-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoyl-CoA + AMP + diphosphate; 12-hydroxy-(5Z,8Z,10E,14Z)-eicosatetraenoate + ATP + CoA = 12-hydroxy-(5Z,8Z,10E,14Z)-eicosatetraenoyl-CoA + AMP + diphosphate; 5-hydroxy-(6E,8Z,11Z,14Z)-eicosatetraenoate + ATP + CoA = 5-hydroxy-(6E,8Z,11Z,14Z)-eicosatetraenoyl-CoA + AMP + diphosphate",
"targ_desciption": "Catalyzes the conversion of long-chain fatty acids to theiractive form acyl-CoA for both synthesis of cellular lipids, anddegradation via beta-oxidation (PubMed:22633490, PubMed:24269233).Plays an important role in fatty acid metabolism in brain and the acyl-CoAs produced may be utilized exclusively for the synthesis of thebrain lipid. {ECO:0000269|PubMed:22633490,ECO:0000269|PubMed:24269233}.",
"references": []
},
{
"targ_id": "T027006",
"parent_targ_id": "T027",
"full_name": "Long-chain-fatty-acid--CoA ligase ACSBG1",
"abbrev": "hBG1",
"protein_names": "Acyl-CoA synthetase bubblegum family member 1",
"related_func_ids": "F02060101",
"category": "enzyme",
"subcategories": "Ligase",
"Uniport_ID": "Q96GR2",
"Uniprot_name": "ACBG1_HUMAN",
"EC_numbers": "6.2.1.3",
"gene_symbol": "ACSBG1",
"gene_synonyms": "",
"gene_synonyms_links": "",
"gene_name": "",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "",
"omim_id": "",
"genecard_link": "",
"locs": "Cytoplasm; Cytoplasmic vesicle; Microsome; Endoplasmic reticulum; Cell membrane",
"reaction": "a long-chain fatty acid + ATP + CoA = a long-chain fatty acyl-CoA + AMP + diphosphate; (E)-hexadec-2-enoate + ATP + CoA = (2E)-hexadecenoyl-CoA + AMP+ diphosphate; ATP + CoA + hexadecanoate = AMP + diphosphate + hexadecanoyl-CoA",
"targ_desciption": "Catalyzes the conversion of fatty acids such as long-chainand very long-chain fatty acids to their active form acyl-CoAs for bothsynthesis of cellular lipids, and degradation via beta-oxidation(PubMed:12975357, PubMed:24269233, PubMed:10954726). Can activatediverse saturated, monosaturated and polyunsaturated fatty acids(PubMed:10954726). {ECO:0000269|PubMed:10954726,ECO:0000269|PubMed:12975357, ECO:0000269|PubMed:24269233}.",
"references": []
},
{
"targ_id": "T027007",
"parent_targ_id": "T027",
"full_name": "Long-chain-fatty-acid--CoA ligase ACSBG2",
"abbrev": "ACSBG2",
"protein_names": "Acyl-CoA synthetase bubblegum family member 2",
"related_func_ids": "F02060101",
"category": "enzyme",
"subcategories": "Ligase",
"Uniport_ID": "Q5FVE4",
"Uniprot_name": "ACBG2_HUMAN",
"EC_numbers": "6.2.1.3",
"gene_symbol": "ACSBG2",
"gene_synonyms": "",
"gene_synonyms_links": "",
"gene_name": "",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "",
"omim_id": "",
"genecard_link": "",
"locs": "Cytoplasm; Membrane; Peripheral membrane protein",
"reaction": "a long-chain fatty acid + ATP + CoA = a long-chain fatty acyl-CoA + AMP + diphosphate; (5Z,8Z,11Z,14Z)-eicosatetraenoate + ATP + CoA =(5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoA + AMP + diphosphate; ATP + CoA + hexadecanoate = AMP + diphosphate + hexadecanoyl-CoA; (9Z)-octadecenoate + ATP + CoA = (9Z)-octadecenoyl-CoA + AMP +diphosphate; (9Z,12Z)-octadecadienoate + ATP + CoA = (9Z,12Z)-octadecadienoyl-CoA + AMP + diphosphate; ATP + CoA + tetracosanoate = AMP + diphosphate +tetracosanoyl-CoA",
"targ_desciption": "Catalyzes the conversion of fatty acids such as long chainand very long-chain fatty acids to their active form acyl-CoAs for bothsynthesis of cellular lipids, and degradation via beta-oxidation. Canactivate diverse saturated, monosaturated and polyunsaturated fattyacids (PubMed:16371355, PubMed:16762313). Has increased ability toactivate oleic and linoleic acid (PubMed:16371355). May play a role inspermatogenesis (PubMed:15685348). {ECO:0000269|PubMed:15685348,ECO:0000269|PubMed:16371355, ECO:0000269|PubMed:16762313}.",
"references": []
},
{
"targ_id": "T027008",
"parent_targ_id": "T027",
"full_name": "Very long-chain acyl-CoA synthetase",
"abbrev": "VLACS",
"protein_names": "a long-chain fatty acid + ATP + CoA = a long-chain fatty acyl-CoA + AMP + diphosphate",
"related_func_ids": "F02060101",
"category": "enzyme",
"subcategories": "Ligase",
"Uniport_ID": "O14975",
"Uniprot_name": "S27A2_HUMAN",
"EC_numbers": "6.2.1.-; 6.2.1.15; 6.2.1.3; 6.2.1.24; 6.2.1.7",
"gene_symbol": "SLC27A2",
"gene_synonyms": "ACSVL1, FACVL1, FATP2, hFACVL1, HsT17226, VLACS, VLCS",
"gene_synonyms_links": "",
"gene_name": "Solute carrier family 27 member 2",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000140284",
"omim_id": "",
"genecard_link": "",
"locs": "Endoplasmic reticulum membrane; Multi-pass membrane protein; Peroxisome membrane; Peripheral membrane protein; Cell membrane; Multi-pass membrane protein; Microsome",
"reaction": "a long-chain fatty acid + ATP + CoA = a long-chain fatty acyl-CoA + AMP + diphosphate; (5Z,8Z,11Z,14Z)-eicosatetraenoate + ATP + CoA =(5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoA + AMP + diphosphate; 3,7,11,15-tetramethylhexadecanoate + ATP + CoA = AMP +diphosphate + phytanoyl-CoA; (25R)-3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-26-oate+ ATP + CoA = (25R)-3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-26-oyl-CoA + AMP + diphosphate; a very long-chain fatty acid + ATP + CoA = a very long-chainfatty acyl-CoA + AMP + diphosphate; a fatty acid(in) = a fatty acid(out); ATP + CoA + tetracosanoate = AMP + diphosphate +tetracosanoyl-CoA; (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoate + ATP + CoA =(4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoyl-CoA + AMP + diphosphate; (9Z,12Z,15Z)-octadecatrienoate + ATP + CoA = (9Z,12Z,15Z)-octadecatrienoyl-CoA + AMP + diphosphate; ATP + CoA + hexadecanoate = AMP + diphosphate + hexadecanoyl-CoA; 2,6,10,14-tetramethylpentadecanoate + ATP + CoA = AMP +diphosphate + pristanoyl-CoA; (E)-hexadec-2-enoate + ATP + CoA = (2E)-hexadecenoyl-CoA + AMP+ diphosphate",
"targ_desciption": "Acyl CoA synthetase that activates long-chain and very long-chain fatty acids (VLCFAs) by catalyzing the formation of fatty acyl-CoA (PubMed:10198260, PubMed:10749848, PubMed:11980911). Can alsoactivate branched-chain fatty acids such as phytanic acid and pristanicacid (PubMed:10198260). Does not activate C24 bile acids, cholate andchenodeoxycholate (PubMed:11980911). In vitro, activates 3-alpha,7-alpha,12-alpha-trihydroxy-5-beta-cholestanate (THCA), the C27 precursorof cholic acid deriving from the de novo synthesis from cholesterol(PubMed:11980911). Exhibits long-chain fatty acids (LCFA) transportactivity and plays an important role in hepatic fatty acid uptake(PubMed:20530735). {ECO:0000269|PubMed:10198260,ECO:0000269|PubMed:10749848, ECO:0000269|PubMed:11980911,ECO:0000269|PubMed:20530735}.; [Isoform 1]: Exhibits both long-chain fatty acids (LCFA)transport activity and acyl CoA synthetase towards very long-chainfatty acids (PubMed:21768100). Shows a preference for generating CoAderivatives of n-3 fatty acids, which are preferentially traffickedinto phosphatidylinositol (PubMed:21768100).{ECO:0000269|PubMed:21768100}.; [Isoform 2]: Exhibits long-chain fatty acids (LCFA) transportactivity but lacks acyl CoA synthetase towards very long-chain fattyacids. {ECO:0000269|PubMed:21768100}.",
"references": []
},
{
"targ_id": "T028",
"parent_targ_id": "T",
"full_name": "Short-chain specific acyl-CoA dehydrogenase, mitochondrial",
"abbrev": "SCAD",
"protein_names": "Short-chain specific acyl-CoA dehydrogenase, mitochondrial (SCAD) (EC 1.3.8.1) (Butyryl-CoA dehydrogenase)",
"related_func_ids": "F02060101",
"category": "enzyme",
"subcategories": "Oxidoreductase",
"Uniport_ID": "P16219",
"Uniprot_name": "ACADS_HUMAN",
"EC_numbers": "1.3.8.1",
"gene_symbol": "ACADS",
"gene_synonyms": "ACAD3, SCAD",
"gene_synonyms_links": "",
"gene_name": "Acyl-CoA dehydrogenase short chain",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000122971",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion matrix",
"reaction": "a short-chain 2,3-saturated fatty acyl-CoA + H(+) + oxidized[electron-transfer flavoprotein] = a short-chain (2E)-enoyl-CoA +reduced [electron-transfer flavoprotein]; butanoyl-CoA + H(+) + oxidized [electron-transferflavoprotein] = (2E)-butenoyl-CoA + reduced [electron-transferflavoprotein]; H(+) + oxidized [electron-transfer flavoprotein] + pentanoyl-CoA = (2E)-pentenoyl-CoA + reduced [electron-transfer flavoprotein]; H(+) + hexanoyl-CoA + oxidized [electron-transferflavoprotein] = (2E)-hexenoyl-CoA + reduced [electron-transferflavoprotein]",
"targ_desciption": "Short-chain specific acyl-CoA dehydrogenase is one of theacyl-CoA dehydrogenases that catalyze the first step of mitochondrialfatty acid beta-oxidation, an aerobic process breaking down fatty acidsinto acetyl-CoA and allowing the production of energy from fats (Bysimilarity). The first step of fatty acid beta-oxidation consists inthe removal of one hydrogen from C-2 and C-3 of the straight-chainfatty acyl-CoA thioester, resulting in the formation of trans-2-enoyl-CoA (By similarity). Among the different mitochondrial acyl-CoAdehydrogenases, short-chain specific acyl-CoA dehydrogenase actsspecifically on acyl-CoAs with saturated 4 to 6 carbons long primarychains (PubMed:21237683, PubMed:11134486).{ECO:0000250|UniProtKB:P15651, ECO:0000269|PubMed:11134486,ECO:0000269|PubMed:21237683}.",
"references": [
"RC00854",
"RC00855",
"RC00856",
"RC00857",
"RC00858",
"RC00859",
"RC00860"
]
},
{
"targ_id": "T029",
"parent_targ_id": "T",
"full_name": "Medium-chain specific acyl-CoA dehydrogenase, mitochondrial",
"abbrev": "MCAD",
"protein_names": "Medium-chain specific acyl-CoA dehydrogenase, mitochondrial (MCAD) (EC 1.3.8.7)",
"related_func_ids": "F02060101",
"category": "enzyme",
"subcategories": "Oxidoreductase",
"Uniport_ID": "P11310",
"Uniprot_name": "ACADM_HUMAN",
"EC_numbers": "1.3.8.7",
"gene_symbol": "ACADM",
"gene_synonyms": "ACAD1, MCAD, MCADH",
"gene_synonyms_links": "",
"gene_name": "Acyl-CoA dehydrogenase medium chain",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000117054",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion matrix",
"reaction": "a medium-chain 2,3-saturated fatty acyl-CoA + H(+) + oxidized[electron-transfer flavoprotein] = a medium-chain trans-(2E)-enoyl-CoA + reduced [electron-transfer flavoprotein]",
"targ_desciption": "Acyl-CoA dehydrogenase specific for acyl chain lengths of 4to 16 that catalyzes the initial step of fatty acid beta-oxidation.Utilizes the electron transfer flavoprotein (ETF) as an electronacceptor to transfer electrons to the main mitochondrial respiratorychain via ETF-ubiquinone oxidoreductase (ETF dehydrogenase).{ECO:0000269|PubMed:25416781}.",
"references": [
"RC00847",
"RC00848",
"RC00849",
"RC00850",
"RC00851",
"RC00852",
"RC00853"
]
},
{
"targ_id": "T030",
"parent_targ_id": "T",
"full_name": "Long-chain specific acyl-CoA dehydrogenase, mitochondrial",
"abbrev": "LCAD",
"protein_names": "Long-chain specific acyl-CoA dehydrogenase, mitochondrial (LCAD) (EC 1.3.8.8)",
"related_func_ids": "F02060101",
"category": "enzyme",
"subcategories": "Oxidoreductase",
"Uniport_ID": "P28330",
"Uniprot_name": "ACADL_HUMAN",
"EC_numbers": "1.3.8.8",
"gene_symbol": "ACADL",
"gene_synonyms": "ACAD4, LCAD",
"gene_synonyms_links": "",
"gene_name": "Acyl-CoA dehydrogenase long chain",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000115361",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion matrix",
"reaction": "a long-chain 2,3-saturated fatty acyl-CoA + H(+) + oxidized[electron-transfer flavoprotein] = a long-chain (2E)-enoyl-CoA +reduced [electron-transfer flavoprotein]; H(+) + hexanoyl-CoA + oxidized [electron-transferflavoprotein] = (2E)-hexenoyl-CoA + reduced [electron-transferflavoprotein]; H(+) + octanoyl-CoA + oxidized [electron-transferflavoprotein] = (2E)-octenoyl-CoA + reduced [electron-transferflavoprotein]; decanoyl-CoA + H(+) + oxidized [electron-transferflavoprotein] = (2E)-decenoyl-CoA + reduced [electron-transferflavoprotein]; dodecanoyl-CoA + H(+) + oxidized [electron-transferflavoprotein] = (2E)-dodecenoyl-CoA + reduced [electron-transferflavoprotein]; H(+) + oxidized [electron-transfer flavoprotein] +tetradecanoyl-CoA = (2E)-tetradecenoyl-CoA + reduced [electron-transfer flavoprotein]; H(+) + hexadecanoyl-CoA + oxidized [electron-transferflavoprotein] = (2E)-hexadecenoyl-CoA + reduced [electron-transferflavoprotein]; H(+) + octadecanoyl-CoA + oxidized [electron-transferflavoprotein] = (2E)-octadecenoyl-CoA + reduced [electron-transferflavoprotein]; eicosanoyl-CoA + H(+) + oxidized [electron-transferflavoprotein] = (2E)-eicosenoyl-CoA + reduced [electron-transferflavoprotein]; docosanoyl-CoA + H(+) + oxidized [electron-transferflavoprotein] = (2E)-docosenoyl-CoA + reduced [electron-transferflavoprotein]; H(+) + oxidized [electron-transfer flavoprotein] +tetracosanoyl-CoA = (2E)-tetracosenoyl-CoA + reduced [electron-transfer flavoprotein]; (5E)-tetradecenoyl-CoA + H(+) + oxidized [electron-transferflavoprotein] = (2E,5E)-tetradecadienoyl-CoA + reduced [electron-transfer flavoprotein]; (5Z)-tetradecenoyl-CoA + H(+) + oxidized [electron-transferflavoprotein] = (2E,5Z)-tetradecadienoyl-CoA + reduced [electron-transfer flavoprotein]; (9Z)-octadecenoyl-CoA + H(+) + oxidized [electron-transferflavoprotein] = (2E,9Z)-octadecadienoyl-CoA + reduced [electron-transfer flavoprotein]",
"targ_desciption": "Long-chain specific acyl-CoA dehydrogenase is one of theacyl-CoA dehydrogenases that catalyze the first step of mitochondrialfatty acid beta-oxidation, an aerobic process breaking down fatty acidsinto acetyl-CoA and allowing the production of energy from fats (Bysimilarity). The first step of fatty acid beta-oxidation consists inthe removal of one hydrogen from C-2 and C-3 of the straight-chainfatty acyl-CoA thioester, resulting in the formation of trans-2-enoyl-CoA (By similarity). Among the different mitochondrial acyl-CoAdehydrogenases, long-chain specific acyl-CoA dehydrogenase can act onsaturated and unsaturated acyl-CoAs with 6 to 24 carbons with apreference for 8 to 18 carbons long primary chains (PubMed:8823175,PubMed:21237683). {ECO:0000250|UniProtKB:P15650,ECO:0000269|PubMed:21237683, ECO:0000269|PubMed:8823175}.",
"references": [
"RC00841",
"RC00842",
"RC00843",
"RC00844",
"RC00845",
"RC00846"
]
},
{
"targ_id": "T031",
"parent_targ_id": "T",
"full_name": "Very long-chain specific acyl-CoA dehydrogenase, mitochondrial",
"abbrev": "VLCAD",
"protein_names": "Very long-chain specific acyl-CoA dehydrogenase, mitochondrial (VLCAD) (EC 1.3.8.9)",
"related_func_ids": "F02060101",
"category": "enzyme",
"subcategories": "Oxidoreductase",
"Uniport_ID": "P49748",
"Uniprot_name": "ACADV_HUMAN",
"EC_numbers": "1.3.8.9",
"gene_symbol": "ACADVL",
"gene_synonyms": "ACAD6, LCACD, VLCAD",
"gene_synonyms_links": "",
"gene_name": "Acyl-CoA dehydrogenase very long chain",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000072778",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane",
"reaction": "a very-long-chain 2,3-saturated fatty acyl-CoA + H(+) +oxidized [electron-transfer flavoprotein] = a very-long-chain (2E)-enoyl-CoA + reduced [electron-transfer flavoprotein]",
"targ_desciption": "Active toward esters of long-chain and very long chain fattyacids such as palmitoyl-CoA, myristoyl-CoA and stearoyl-CoA. Canaccommodate substrate acyl chain lengths as long as 24 carbons, butshows little activity for substrates of less than 12 carbons.{ECO:0000269|PubMed:18227065}.",
"references": []
},
{
"targ_id": "T032",
"parent_targ_id": "T",
"full_name": "Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial",
"abbrev": "SBCAD",
"protein_names": "Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial (SBCAD) (EC 1.3.8.5) (2-methyl branched chain acyl-CoA dehydrogenase) (2-MEBCAD) (2-methylbutyryl-coenzyme A dehydrogenase) (2-methylbutyryl-CoA dehydrogenase)",
"related_func_ids": "F02060101",
"category": "enzyme",
"subcategories": "Oxidoreductase",
"Uniport_ID": "P45954",
"Uniprot_name": "ACDSB_HUMAN",
"EC_numbers": "1.3.8.5",
"gene_symbol": "ACADSB",
"gene_synonyms": "ACAD7, SBCAD",
"gene_synonyms_links": "",
"gene_name": "Acyl-CoA dehydrogenase short/branched chain",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000196177",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion matrix",
"reaction": "A + a 2,3-saturated acyl-CoA = a 2,3-dehydroacyl-CoA + AH2; 2-methylbutanoyl-CoA + H(+) + oxidized [electron-transferflavoprotein] = (2E)-2-methylbutenoyl-CoA + reduced [electron-transfer flavoprotein]",
"targ_desciption": "Has greatest activity toward short branched chain acyl-CoA derivative such as (s)-2-methylbutyryl-CoA, isobutyryl-CoA, and 2-methylhexanoyl-CoA as well as toward short straight chain acyl-CoAssuch as butyryl-CoA and hexanoyl-CoA. Can use valproyl-CoA as substrateand may play a role in controlling the metabolic flux of valproic acidin the development of toxicity of this agent.",
"references": [
"RC00384",
"RC00385",
"RC00386",
"RC00387"
]
},
{
"targ_id": "T033",
"parent_targ_id": "T",
"full_name": "acetate CoA-transferase",
"abbrev": "",
"protein_names": "",
"related_func_ids": "F02060101",
"category": "enzyme",
"subcategories": "transferase",
"Uniport_ID": "",
"Uniprot_name": "ATOA_ECOLI",
"EC_numbers": "2.8.3.8",
"gene_symbol": "",
"gene_synonyms": "",
"gene_synonyms_links": "",
"gene_name": "",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "",
"omim_id": "",
"genecard_link": "",
"locs": "",
"reaction": "",
"targ_desciption": "",
"references": []
},
{
"targ_id": "T034",
"parent_targ_id": "T",
"full_name": "Medium-chain acyl-CoA ligase ACSF2, mitochondrial",
"abbrev": "ACSF2",
"protein_names": "Medium-chain acyl-CoA ligase ACSF2, mitochondrial (EC 6.2.1.2)",
"related_func_ids": "F02060101",
"category": "enzyme",
"subcategories": "Ligase",
"Uniport_ID": "Q96CM8",
"Uniprot_name": "ACSF2_HUMAN",
"EC_numbers": "6.2.1.2",
"gene_symbol": "ACSF2",
"gene_synonyms": "ACSMW, FLJ20920",
"gene_synonyms_links": "",
"gene_name": "Acyl-CoA synthetase family member 2",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000167107",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion",
"reaction": "a medium chain fatty acid + ATP + CoA = a medium-chain fattyacyl-CoA + AMP + diphosphate; ATP + CoA + octanoate = AMP + diphosphate + octanoyl-CoA",
"targ_desciption": "Acyl-CoA synthases catalyze the initial reaction in fattyacid metabolism, by forming a thioester with CoA (PubMed:17762044). Hassome preference toward medium-chain substrates (PubMed:17762044). Playsa role in adipocyte differentiation (PubMed:16380219).{ECO:0000269|PubMed:16380219, ECO:0000269|PubMed:17762044}.",
"references": [
"RC00863",
"RC00864"
]
},
{
"targ_id": "T035",
"parent_targ_id": "T",
"full_name": "Carnitine O-acetyltransferase",
"abbrev": "CAT",
"protein_names": "Carnitine O-acetyltransferase (Carnitine acetylase) (EC 2.3.1.137) (EC 2.3.1.7) (Carnitine acetyltransferase) (CAT) (CrAT)",
"related_func_ids": "F02060101",
"category": "enzyme",
"subcategories": "Acyltransferase, Transferase, Transporter",
"Uniport_ID": "P43155",
"Uniprot_name": "CACP_HUMAN",
"EC_numbers": "2.3.1.137; 2.3.1.7",
"gene_symbol": "CRAT",
"gene_synonyms": "CAT1",
"gene_synonyms_links": "",
"gene_name": "Carnitine O-acetyltransferase",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000095321",
"omim_id": "",
"genecard_link": "",
"locs": "Endoplasmic reticulum; Peroxisome; Mitochondrion inner membrane; Peripheral membrane protein; Matrix side; [Isoform 1]: Mitochondrion; [Isoform 2]: Peroxisome",
"reaction": "(R)-carnitine + acetyl-CoA = CoA + O-acetyl-(R)-carnitine; (R)-carnitine + propanoyl-CoA = CoA + O-propanoyl-(R)-carnitine; (R)-carnitine + butanoyl-CoA = CoA + O-butanoyl-(R)-carnitine; (R)-carnitine + hexanoyl-CoA = CoA + O-hexanoyl-(R)-carnitine; (R)-carnitine + octanoyl-CoA = CoA + O-octanoyl-(R)-carnitine; (R)-carnitine + decanoyl-CoA = CoA + O-decanoyl-(R)-carnitine; (R)-carnitine + 3-methylbutanoyl-CoA = CoA + O-3-methylbutanoyl-(R)-carnitine; (R)-carnitine + 2-methylpropanoyl-CoA = CoA + O-isobutanoyl-(R)-carnitine; (R)-carnitine + 2-methylbutanoyl-CoA = CoA + O-2-methylbutanoyl-(R)-carnitine; (R)-carnitine + acetoacetyl-CoA = CoA + O-3-oxobutanoyl-(R)-carnitine; (R)-carnitine + 3-hydroxybutanoyl-CoA = CoA + O-3-hydroxybutanoyl-(R)-carnitine; (R)-carnitine + 4,8-dimethylnonanoyl-CoA = CoA + O-4,8-dimethylnonanoyl-(R)-carnitine; (R)-carnitine + 2,6-dimethylheptanoyl-CoA = CoA + O-2,6-dimethylheptanoyl-(R)-carnitine",
"targ_desciption": "Catalyzes the reversible transfer of acyl groups fromcarnitine to coenzyme A (CoA) and regulates the acyl-CoA/CoA ratio.Also plays a crucial role in the transport of fatty acids for beta-oxidation (PubMed:15099582, PubMed:29395073). Responsible for thesynthesis of short- and branched-chain acylcarnitines(PubMed:23485643). Active towards some branched-chain amino acidoxidation pathway (BCAAO) intermediates (PubMed:23485643). Trans-2-enoyl-CoAs and 2-methylacyl-CoAs are poor substrates (PubMed:23485643).{ECO:0000269|PubMed:15099582, ECO:0000269|PubMed:23485643,ECO:0000269|PubMed:29395073}.",
"references": []
},
{
"targ_id": "T036",
"parent_targ_id": "T",
"full_name": "Electron transfer flavoprotein",
"abbrev": "ETF",
"protein_names": "",
"related_func_ids": "F02060101; F020103",
"category": "protein",
"subcategories": "flavoprotein",
"Uniport_ID": "",
"Uniprot_name": "",
"EC_numbers": "",
"gene_symbol": "",
"gene_synonyms": "",
"gene_synonyms_links": "",
"gene_name": "",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "",
"omim_id": "",
"genecard_link": "",
"locs": "",
"reaction": "",
"targ_desciption": "",
"references": []
},
{
"targ_id": "T036001",
"parent_targ_id": "T036",
"full_name": "Electron transfer flavoprotein subunit alpha, mitochondrial",
"abbrev": "Alpha-ETF",
"protein_names": "Electron transfer flavoprotein subunit alpha, mitochondrial (Alpha-ETF)",
"related_func_ids": "F02060101; F020103",
"category": "protein subunit",
"subcategories": "flavoprotein subunit",
"Uniport_ID": "P13804",
"Uniprot_name": "ETFA_HUMAN",
"EC_numbers": "",
"gene_symbol": "ETFA",
"gene_synonyms": "EMA, GA2, MADD",
"gene_synonyms_links": "",
"gene_name": "Electron transfer flavoprotein alpha subunit",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000140374",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion matrix",
"reaction": "",
"targ_desciption": "Heterodimeric electron transfer flavoprotein that acceptselectrons from several mitochondrial dehydrogenases, including acyl-CoAdehydrogenases, glutaryl-CoA and sarcosine dehydrogenase(PubMed:27499296, PubMed:15159392, PubMed:15975918, PubMed:9334218,PubMed:10356313). It transfers the electrons to the main mitochondrialrespiratory chain via ETF-ubiquinone oxidoreductase (ETF dehydrogenase)(PubMed:9334218). Required for normal mitochondrial fatty acidoxidation and normal amino acid metabolism (PubMed:12815589,PubMed:1882842, PubMed:1430199). {ECO:0000269|PubMed:10356313,ECO:0000269|PubMed:12815589, ECO:0000269|PubMed:1430199,ECO:0000269|PubMed:15159392, ECO:0000269|PubMed:15975918,ECO:0000269|PubMed:27499296, ECO:0000269|PubMed:9334218,ECO:0000303|PubMed:17941859, ECO:0000305|PubMed:1882842}.",
"references": []
},
{
"targ_id": "T036002",
"parent_targ_id": "T036",
"full_name": "Electron transfer flavoprotein beta subunit lysine methyltransferase",
"abbrev": "ETFB-KMT",
"protein_names": "Electron transfer flavoprotein beta subunit lysine methyltransferase (EC 2.1.1.-) (ETFB lysine methyltransferase) (ETFB-KMT) (Protein N-lysine methyltransferase METTL20)",
"related_func_ids": "F02060101; F020103",
"category": "protein subunit",
"subcategories": "flavoprotein subunit, Methyltransferase, Transferase",
"Uniport_ID": "Q8IXQ9",
"Uniprot_name": "ETKMT_HUMAN",
"EC_numbers": "2.1.1.-",
"gene_symbol": "ETFBKMT",
"gene_synonyms": "C12orf72, DKFZp451L235, METTL20, MGC50559",
"gene_synonyms_links": "",
"gene_name": "Electron transfer flavoprotein beta subunit lysine methyltransferase",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000139160",
"omim_id": "",
"genecard_link": "",
"locs": "Cytoplasm; Mitochondrion matrix;",
"reaction": "",
"targ_desciption": "Protein-lysine methyltransferase that selectivelytrimethylates the flavoprotein ETFB in mitochondria (PubMed:25023281,PubMed:25416781). Thereby, may negatively regulate the function of ETFBin electron transfer from Acyl-CoA dehydrogenases to the mainrespiratory chain (PubMed:25416781). {ECO:0000269|PubMed:25023281,ECO:0000269|PubMed:25416781}.",
"references": []
},
{
"targ_id": "T037",
"parent_targ_id": "T",
"full_name": "Electron transfer flavoprotein-ubiquinone oxidoreductase, mitochondrial",
"abbrev": "ETF-QO",
"protein_names": "Electron transfer flavoprotein-ubiquinone oxidoreductase, mitochondrial (ETF-QO) (ETF-ubiquinone oxidoreductase) (EC 1.5.5.1) (Electron-transferring-flavoprotein dehydrogenase) (ETF dehydrogenase)",
"related_func_ids": "F02060101; F020103; F05",
"category": "enzyme",
"subcategories": "Oxidoreductase",
"Uniport_ID": "Q16134",
"Uniprot_name": "ETFD_HUMAN",
"EC_numbers": "1.5.5.1",
"gene_symbol": "ETFDH",
"gene_synonyms": "ETFQO",
"gene_synonyms_links": "",
"gene_name": "Electron transfer flavoprotein dehydrogenase",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000171503",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane",
"reaction": "a ubiquinone + reduced [electron-transfer flavoprotein] = aubiquinol + H(+) + oxidized [electron-transfer flavoprotein]",
"targ_desciption": "Accepts electrons from ETF and reduces ubiquinone.",
"references": []
},
{
"targ_id": "T038",
"parent_targ_id": "T",
"full_name": "Fatty acid 2-hydroxylase",
"abbrev": "Fatty acid alpha-hydroxylase",
"protein_names": "Fatty acid 2-hydroxylase (EC 1.14.18.-) (Fatty acid alpha-hydroxylase) (Fatty acid hydroxylase domain-containing protein 1)",
"related_func_ids": "F02060101; F060303",
"category": "enzyme",
"subcategories": "Oxidoreductase",
"Uniport_ID": "Q7L5A8",
"Uniprot_name": "FA2H_HUMAN",
"EC_numbers": "1.14.18.-",
"gene_symbol": "FA2H FAAH FAXDC1",
"gene_synonyms": "",
"gene_synonyms_links": "",
"gene_name": "",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "",
"omim_id": "",
"genecard_link": "",
"locs": "Endoplasmic reticulum membrane; Multi-pass membrane protein; Microsome membrane; Multi-pass membrane protein",
"reaction": "2 [Fe(II)-cytochrome b5] + a 1,2-saturated fatty acid + 2 H(+)+ O2 = 2 [Fe(III)-cytochrome b5] + a (R)-2-hydroxy fatty acid + H2O; 2 [Fe(II)-cytochrome b5] + 2 H(+) + hexadecanoate + O2 = (R)-2-hydroxyhexadecanoate + 2 [Fe(III)-cytochrome b5] + H2O; 2 [Fe(II)-cytochrome b5] + 2 H(+) + O2 + octadecanoate = 2-hydroxyoctadecanoate + 2 [Fe(III)-cytochrome b5] + H2O; 2 [Fe(II)-cytochrome b5] + docosanoate + 2 H(+) + O2 = 2-hydroxydocosanoate + 2 [Fe(III)-cytochrome b5] + H2O; 2 [Fe(II)-cytochrome b5] + 2 H(+) + O2 + tetracosanoate = 2-hydroxytetracosanoate + 2 [Fe(III)-cytochrome b5] + H2O",
"targ_desciption": "Catalyzes the hydroxylation of free fatty acids at the C-2position to produce 2-hydroxy fatty acids, which are building blocks ofsphingolipids and glycosphingolipids common in neural tissue andepidermis (PubMed:15337768, PubMed:15863841, PubMed:17355976,PubMed:22517924). FA2H is stereospecific for the production of (R)-2-hydroxy fatty acids (PubMed:22517924). Plays an essential role in thesynthesis of galactosphingolipids of the myelin sheath (By similarity).Responsible for the synthesis of sphingolipids and glycosphingolipidsinvolved in the formation of epidermal lamellar bodies critical forskin permeability barrier (PubMed:17355976). Participates in thesynthesis of glycosphingolipids and a fraction of type II wax diestersin sebaceous gland, specifically regulating hair follicle homeostasis(By similarity). Involved in the synthesis of sphingolipids of plasmamembrane rafts, controlling lipid raft mobility and trafficking ofraft-associated proteins (By similarity).{ECO:0000250|UniProtKB:Q5MPP0, ECO:0000269|PubMed:15337768,ECO:0000269|PubMed:15863841, ECO:0000269|PubMed:17355976,ECO:0000269|PubMed:22517924}.",
"references": []
},
{
"targ_id": "T039",
"parent_targ_id": "T",
"full_name": "3-hydroxyacyl-CoA dehydrogenase type-2",
"abbrev": "MHBD",
"protein_names": "3-hydroxyacyl-CoA dehydrogenase type-2 (EC 1.1.1.35) (17-beta-hydroxysteroid dehydrogenase 10) (17-beta-HSD 10) (EC 1.1.1.51) (2-methyl-3-hydroxybutyryl-CoA dehydrogenase) (MHBD) (3-hydroxy-2-methylbutyryl-CoA dehydrogenase) (EC 1.1.1.178) (3-hydroxyacyl-CoA dehydrogenase type II) (Endoplasmic reticulum-associated amyloid beta-peptide-binding protein) (Mitochondrial ribonuclease P protein 2) (Mitochondrial RNase P protein 2) (Short chain dehydrogenase/reductase family 5C member 1) (Short-chain type dehydrogenase/reductase XH98G2) (Type II HADH)",
"related_func_ids": "F02060101; F060303; F03",
"category": "enzyme",
"subcategories": "Oxidoreductase",
"Uniport_ID": "Q99714",
"Uniprot_name": "HCD2_HUMAN",
"EC_numbers": "1.1.1.35; 1.1.1.51; 1.1.1.178",
"gene_symbol": "HSD17B10",
"gene_synonyms": "17b-HSD10, ABAD, CAMR, ERAB, HADH2, MHBD, MRPP2, MRXS10, SDR5C1",
"gene_synonyms_links": "",
"gene_name": "Hydroxysteroid 17-beta dehydrogenase 10",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000072506",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion",
"reaction": "(2S,3S)-3-hydroxy-2-methylbutanoyl-CoA + NAD(+) = 2-methyl-3-oxobutanoyl-CoA + H(+) + NADH; NAD(+) + testosterone = androst-4-ene-3,17-dione + H(+) +NADH; NADP(+) + testosterone = androst-4-ene-3,17-dione + H(+) +NADPH; a (3S)-hydroxyacyl-CoA + NAD(+) = a 3-oxoacyl-CoA + H(+) +NADH",
"targ_desciption": "Mitochondrial dehydrogenase that catalyzes the beta-oxidationat position 17 of androgens and estrogens and has 3-alpha-hydroxysteroid dehydrogenase activity with androsterone(PubMed:9553139, PubMed:23042678, PubMed:12917011, PubMed:18996107,PubMed:25925575, PubMed:28888424). Catalyzes the third step in thebeta-oxidation of fatty acids (PubMed:9553139, PubMed:12917011,PubMed:18996107, PubMed:25925575, PubMed:28888424). Carries outoxidative conversions of 7-alpha-OH and 7-beta-OH bile acids(PubMed:12917011). Also exhibits 20-beta-OH and 21-OH dehydrogenaseactivities with C21 steroids (PubMed:12917011). By interacting withintracellular amyloid-beta, it may contribute to the neuronaldysfunction associated with Alzheimer disease (AD) (PubMed:9338779).Essential for structural and functional integrity of mitochondria(PubMed:20077426). {ECO:0000269|PubMed:12917011,ECO:0000269|PubMed:18996107, ECO:0000269|PubMed:20077426,ECO:0000269|PubMed:23042678, ECO:0000269|PubMed:25925575,ECO:0000269|PubMed:26950678, ECO:0000269|PubMed:28888424,ECO:0000269|PubMed:9338779, ECO:0000269|PubMed:9553139}.; In addition to mitochondrial dehydrogenase activity,moonlights as a component of mitochondrial ribonuclease P, a complexthat cleaves tRNA molecules in their 5'-ends (PubMed:18984158,PubMed:24549042, PubMed:25925575, PubMed:26950678, PubMed:28888424).Together with HSD17B10/MRPP2, forms a subcomplex of the mitochondrialribonuclease P, named MRPP1-MRPP2 subcomplex, which displays functionsthat are independent of the ribonuclease P activity (PubMed:23042678,PubMed:29040705). The MRPP1-MRPP2 subcomplex catalyzes the formation ofN(1)-methylguanine and N(1)-methyladenine at position 9 (m1G9 and m1A9,respectively) in tRNAs. HSD17B10/MRPP2 acting as a non-catalyticsubunit (PubMed:23042678, PubMed:25925575, PubMed:28888424). The MRPP1-MRPP2 subcomplex also acts as a tRNA maturation platform: following 5'-end cleavage by the mitochondrial ribonuclease P complex, the MRPP1-MRPP2 subcomplex enhances the efficiency of 3'-processing catalyzed byELAC2, retains the tRNA product after ELAC2 processing and presents thenascent tRNA to the mitochondrial CCA tRNA nucleotidyltransferase TRNT1enzyme (PubMed:29040705). {ECO:0000269|PubMed:18984158,ECO:0000269|PubMed:23042678, ECO:0000269|PubMed:24549042,ECO:0000269|PubMed:25925575, ECO:0000269|PubMed:26950678,ECO:0000269|PubMed:28888424, ECO:0000269|PubMed:29040705}.",
"references": []
},
{
"targ_id": "T040",
"parent_targ_id": "T",
"full_name": "Pyrroline-5-carboxylate reductase 1, mitochondrial",
"abbrev": "P5CR 1",
"protein_names": "Pyrroline-5-carboxylate reductase 1, mitochondrial (P5C reductase 1) (P5CR 1) (EC 1.5.1.2)",
"related_func_ids": "F02060101; F01",
"category": "enzyme",
"subcategories": "",
"Uniport_ID": "P32322",
"Uniprot_name": "P5CR1_HUMAN",
"EC_numbers": "1.5.1.2",
"gene_symbol": "PYCR1",
"gene_synonyms": "P5C",
"gene_synonyms_links": "",
"gene_name": "Pyrroline-5-carboxylate reductase 1",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000183010",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion",
"reaction": "L-proline + NADP(+) = 1-pyrroline-5-carboxylate + 2 H(+) +NADPH; L-proline + NAD(+) = 1-pyrroline-5-carboxylate + 2 H(+) +NADH",
"targ_desciption": "Housekeeping enzyme that catalyzes the last step in prolinebiosynthesis. Can utilize both NAD and NADP, but has higher affinityfor NAD. Involved in the cellular response to oxidative stress.{ECO:0000269|PubMed:16730026, ECO:0000269|PubMed:19648921}.",
"references": []
},
{
"targ_id": "T041",
"parent_targ_id": "T",
"full_name": "Tricarboxylate transport protein, mitochondrial",
"abbrev": "CTP",
"protein_names": "Tricarboxylate transport protein, mitochondrial (Citrate transport protein) (CTP) (Solute carrier family 25 member 1) (Tricarboxylate carrier protein)",
"related_func_ids": "F02060102; F020402; F02110114",
"category": "protein",
"subcategories": "transporter",
"Uniport_ID": "P53007",
"Uniprot_name": "TXTP_HUMAN",
"EC_numbers": "",
"gene_symbol": "SLC25A1",
"gene_synonyms": "CTP, SLC20A3",
"gene_synonyms_links": "",
"gene_name": "Solute carrier family 25 member 1",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000100075",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Multi-pass membrane protein",
"reaction": "",
"targ_desciption": "Citrate transporter that mediates the exchange ofmitochondrial citrate for cytosolic malate (PubMed:29031613,PubMed:29238895). Also able to mediate the exchange of citrate forisocitrate, phosphoenolpyruvate, cis- but not trans-aconitate and to alesser extend maleate and succinate (PubMed:29031613). Important forthe bioenergetics of hepatic cells as it provides a carbon source forfatty acid and sterol biosyntheses, and NAD(+) for the glycolyticpathway. Required for proper neuromuscular junction formation(Probable). {ECO:0000269|PubMed:29031613, ECO:0000269|PubMed:29238895,ECO:0000305|PubMed:26870663}.",
"references": []
},
{
"targ_id": "T042",
"parent_targ_id": "T",
"full_name": "Trifunctional enzyme subunit alpha, mitochondrial",
"abbrev": "TP-alpha",
"protein_names": "Trifunctional enzyme subunit alpha, mitochondrial (78 kDa gastrin-binding protein) (Monolysocardiolipin acyltransferase) (EC 2.3.1.-) (TP-alpha) [Includes: Long-chain enoyl-CoA hydratase (EC 4.2.1.17); Long chain 3-hydroxyacyl-CoA dehydrogenase (EC 1.1.1.211)]",
"related_func_ids": "F02060101",
"category": "enzyme",
"subcategories": "Lyase, Multifunctional enzyme, Oxidoreductase, Transferase",
"Uniport_ID": "P40939",
"Uniprot_name": "ECHA_HUMAN",
"EC_numbers": "2.3.1.-; 4.2.1.17; 1.1.1.211",
"gene_symbol": "HADHA",
"gene_synonyms": "GBP, LCEH, LCHAD, MTPA",
"gene_synonyms_links": "",
"gene_name": "Hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alpha",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000084754",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion; Mitochondrion inner membrane;",
"reaction": "a (3S)-hydroxyacyl-CoA = a (2E)-enoyl-CoA + H2O; a 4-saturated-(3S)-hydroxyacyl-CoA = a (3E)-enoyl-CoA + H2O; (3S)-hydroxyoctanoyl-CoA = (2E)-octenoyl-CoA + H2O; (3S)-hydroxydodecanoyl-CoA = (2E)-dodecenoyl-CoA + H2O; (3S)-hydroxyhexadecanoyl-CoA = (2E)-hexadecenoyl-CoA + H2O; a long-chain (3S)-hydroxy fatty acyl-CoA + NAD(+) = a long-chain 3-oxo-fatty acyl-CoA + H(+) + NADH; (3S)-hydroxyoctanoyl-CoA + NAD(+) = 3-oxooctanoyl-CoA + H(+) +NADH; (3S)-hydroxydecanoyl-CoA + NAD(+) = 3-oxodecanoyl-CoA + H(+) +NADH; (3S)-hydroxydodecanoyl-CoA + NAD(+) = 3-oxododecanoyl-CoA +H(+) + NADH; (3S)-hydroxytetradecanoyl-CoA + NAD(+) = 3-oxotetradecanoyl-CoA + H(+) + NADH; (3S)-hydroxyhexadecanoyl-CoA + NAD(+) = 3-oxohexadecanoyl-CoA+ H(+) + NADH; 1'-[1,2-di-(9Z,12Z-octadecadienoyl)-sn-glycero-3-phospho]-3'-[1-(9Z,12Z-octadecadienoyl)-sn-glycero-3-phospho]-glycerol +hexadecanoyl-CoA = 1'-[1,2-di-(9Z,12Z-octadecadienoyl)-sn-glycero-3-phospho]-3'-[1-(9Z,12Z-octadecadienoyl)-2-hexadecanoyl-sn-glycero-3-phospho]-glycerol + CoA; (9Z)-octadecenoyl-CoA + 1'-[1,2-di-(9Z,12Z-octadecadienoyl)-sn-glycero-3-phospho]-3'-[1-(9Z,12Z-octadecadienoyl)-sn-glycero-3-phospho]-glycerol = 1'-[1,2-di-(9Z,12Z-octadecadienoyl)-sn-glycero-3-phospho]-3'-[1-(9Z,12Z-octadecadienoyl)-2-(9Z-octadecenoyl)-sn-glycero-3-phospho]-glycerol + CoA; (9Z,12Z)-octadecadienoyl-CoA + 1'-[1,2-di-(9Z,12Z-octadecadienoyl)-sn-glycero-3-phospho]-3'-[1-(9Z,12Z-octadecadienoyl)-sn-glycero-3-phospho]-glycerol = 1',3'-bis-[1,2-di-(9Z,12Z-octadecadienoyl)-sn-glycero-3-phospho]-glycerol + CoA",
"targ_desciption": "Mitochondrial trifunctional enzyme catalyzes the last threeof the four reactions of the mitochondrial beta-oxidation pathway(PubMed:8135828, PubMed:1550553, PubMed:29915090, PubMed:30850536). Themitochondrial beta-oxidation pathway is the major energy-producingprocess in tissues and is performed through four consecutive reactionsbreaking down fatty acids into acetyl-CoA (PubMed:29915090). Among theenzymes involved in this pathway, the trifunctional enzyme exhibitsspecificity for long-chain fatty acids (PubMed:30850536). Mitochondrialtrifunctional enzyme is a heterotetrameric complex composed of twoproteins, the trifunctional enzyme subunit alpha/HADHA described herecarries the 2,3-enoyl-CoA hydratase and the 3-hydroxyacyl-CoAdehydrogenase activities while the trifunctional enzyme subunitbeta/HADHB bears the 3-ketoacyl-CoA thiolase activity (PubMed:8135828,PubMed:29915090, PubMed:30850536). Independently of the subunit beta,the trifunctional enzyme subunit alpha/HADHA also has amonolysocardiolipin acyltransferase activity (PubMed:23152787). Itacylates monolysocardiolipin into cardiolipin, a major mitochondrialmembrane phospholipid which plays a key role in apoptosis and supportsmitochondrial respiratory chain complexes in the generation of ATP(PubMed:23152787). Allows the acylation of monolysocardiolipin withdifferent acyl-CoA substrates including oleoyl-CoA for which itdisplays the highest activity (PubMed:23152787).{ECO:0000269|PubMed:1550553, ECO:0000269|PubMed:23152787,ECO:0000269|PubMed:29915090, ECO:0000269|PubMed:30850536,ECO:0000269|PubMed:8135828, ECO:0000303|PubMed:29915090,ECO:0000303|PubMed:30850536}.",
"references": []
},
{
"targ_id": "T043",
"parent_targ_id": "T",
"full_name": "Cytochrome P450 11B2, mitochondrial",
"abbrev": "ALDOS",
"protein_names": "Cytochrome P450 11B2, mitochondrial (Aldosterone synthase) (ALDOS) (Aldosterone-synthesizing enzyme) (CYPXIB2) (Corticosterone 18-monooxygenase, CYP11B2) (EC 1.14.15.5) (Cytochrome P-450Aldo) (Cytochrome P-450C18) (Steroid 11-beta-hydroxylase, CYP11B2) (EC 1.14.15.4) (Steroid 18-hydroxylase)",
"related_func_ids": "F020604",
"category": "enzyme",
"subcategories": "Monooxygenase, Oxidoreductase",
"Uniport_ID": "P19099",
"Uniprot_name": "C11B2_HUMAN",
"EC_numbers": "1.14.15.5; 1.14.15.4",
"gene_symbol": "CYP11B2",
"gene_synonyms": "ALDOS, CPN2, CYP11B, CYP11BL, P-450C18, P450aldo",
"gene_synonyms_links": "",
"gene_name": "Cytochrome P450 family 11 subfamily B member 2",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000179142",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Peripheral membrane protein",
"reaction": "a steroid + 2 H(+) + O2 + 2 reduced [adrenodoxin] = an 11beta-hydroxysteroid + H2O + 2 oxidized [adrenodoxin]; 21-hydroxyprogesterone + 2 H(+) + O2 + 2 reduced [adrenodoxin]= corticosterone + H2O + 2 oxidized [adrenodoxin]; corticosterone + 2 H(+) + O2 + 2 reduced [adrenodoxin] = 18-hydroxycorticosterone + H2O + 2 oxidized [adrenodoxin]; 18-hydroxycorticosterone + 2 H(+) + O2 + 2 reduced[adrenodoxin] = aldosterone + 2 H2O + 2 oxidized [adrenodoxin]; 11-deoxycortisol + 2 H(+) + O2 + 2 reduced [adrenodoxin] =cortisol + H2O + 2 oxidized [adrenodoxin]",
"targ_desciption": "A cytochrome P450 monooxygenase that catalyzes thebiosynthesis of adrenal mineralocorticoid aldosterone (PubMed:11856349,PubMed:23322723, PubMed:1594605, PubMed:9814506). Catalyzes threesequential oxidative reactions of 11-deoxycorticosterone/21-hydroxyprogesterone, namely 11-beta hydroxylation followed with twosuccessive oxidations at C18 to yield 18-hydroxy and then 18-aldehydederivatives, resulting in the formation of aldosterone(PubMed:11856349, PubMed:23322723, PubMed:1594605, PubMed:9814506).Mechanistically, uses molecular oxygen inserting one oxygen atom into asubstrate and reducing the second into a water molecule. Two electronsare provided by NADPH via a two-protein mitochondrial transfer systemcomprising flavoprotein FDXR (adrenodoxin/ferredoxin reductase) andnonheme iron-sulfur protein FDX1 or FDX2 (adrenodoxin/ferredoxin)(PubMed:11856349, PubMed:23322723, PubMed:1594605, PubMed:9814506).{ECO:0000269|PubMed:11856349, ECO:0000269|PubMed:1594605,ECO:0000269|PubMed:23322723, ECO:0000269|PubMed:9814506}.",
"references": []
},
{
"targ_id": "T044",
"parent_targ_id": "T",
"full_name": "Phosphatidylserine decarboxylase proenzyme, mitochondrial",
"abbrev": "",
"protein_names": "Phosphatidylserine decarboxylase proenzyme, mitochondrial (EC 4.1.1.65) [Cleaved into: Phosphatidylserine decarboxylase beta chain; Phosphatidylserine decarboxylase alpha chain]",
"related_func_ids": "F021501",
"category": "enzyme",
"subcategories": "Decarboxylase, Lyase",
"Uniport_ID": "Q9UG56",
"Uniprot_name": "PISD_HUMAN",
"EC_numbers": "4.1.1.65",
"gene_symbol": "PISD",
"gene_synonyms": "dJ858B16.2, PSDC",
"gene_synonyms_links": "",
"gene_name": "Phosphatidylserine decarboxylase",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000241878",
"omim_id": "",
"genecard_link": "",
"locs": "[Phosphatidylserine decarboxylase beta chain]:Mitochondrion inner membrane; Single-pass membrane protein; Intermembrane side; [Phosphatidylserine decarboxylase alpha chain]:Mitochondrion inner membrane; Peripheral membrane protein; Intermembrane side;",
"reaction": "a 1,2-diacyl-sn-glycero-3-phospho-L-serine + H(+) = a 1,2-diacyl-sn-glycero-3-phosphoethanolamine + CO2",
"targ_desciption": "Catalyzes the formation of phosphatidylethanolamine (PtdEtn)from phosphatidylserine (PtdSer). Plays a central role in phospholipidmetabolism and in the interorganelle trafficking of phosphatidylserine.{ECO:0000255|HAMAP-Rule:MF_03208}.",
"references": []
},
{
"targ_id": "T045",
"parent_targ_id": "T",
"full_name": "Estradiol 17-beta-dehydrogenase 8",
"abbrev": "17-beta-hydroxysteroid dehydrogenase 8",
"protein_names": "Estradiol 17-beta-dehydrogenase 8 (EC 1.1.1.62) (17-beta-hydroxysteroid dehydrogenase 8) (17-beta-HSD 8) (3-ketoacyl-[acyl-carrier-protein] reductase alpha subunit) (KAR alpha subunit) (3-oxoacyl-[acyl-carrier-protein] reductase) (EC 1.1.1.-) (Protein Ke6) (Ke-6) (Really interesting new gene 2 protein) (Short chain dehydrogenase/reductase family 30C member 1) (Testosterone 17-beta-dehydrogenase 8) (EC 1.1.1.239)",
"related_func_ids": "F02060102",
"category": "enzyme",
"subcategories": "Oxidoreductase",
"Uniport_ID": "Q92506",
"Uniprot_name": "DHB8_HUMAN",
"EC_numbers": "1.1.1.62; 1.1.1.-; 1.1.1.239",
"gene_symbol": "HSD17B8",
"gene_synonyms": "D6S2245E, FABGL, H2-KE6, HKE6, KE6, RING2, SDR30C1",
"gene_synonyms_links": "",
"gene_name": "Hydroxysteroid 17-beta dehydrogenase 8",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000204228",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion matrix",
"reaction": "17beta-estradiol + NAD(+) = estrone + H(+) + NADH; 17beta-estradiol + NADP(+) = estrone + H(+) + NADPH; NAD(+) + testosterone = androst-4-ene-3,17-dione + H(+) +NADH",
"targ_desciption": "NAD-dependent 17-beta-hydroxysteroid dehydrogenase withhighest activity towards estradiol. Has very low activity towardstestosterone (PubMed:17978863). The heterotetramer with CBR4 has NADH-dependent 3-ketoacyl-acyl carrier protein reductase activity, andthereby plays a role in mitochondrial fatty acid biosynthesis(PubMed:19571038, PubMed:25203508). Within the heterotetramer, HSD17B8binds NADH. CBR4 binds NADPD (PubMed:25203508).{ECO:0000269|PubMed:17978863, ECO:0000269|PubMed:19571038,ECO:0000269|PubMed:25203508}.",
"references": []
},
{
"targ_id": "T046",
"parent_targ_id": "T",
"full_name": "Hydroxymethylglutaryl-CoA lyase, mitochondrial",
"abbrev": "HL",
"protein_names": "Hydroxymethylglutaryl-CoA lyase, mitochondrial (HL) (HMG-CoA lyase) (EC 4.1.3.4) (3-hydroxy-3-methylglutarate-CoA lyase)",
"related_func_ids": "F02060201; F03",
"category": "enzyme",
"subcategories": "Lyase",
"Uniport_ID": "P35914",
"Uniprot_name": "HMGCL_HUMAN",
"EC_numbers": "4.1.3.4",
"gene_symbol": "HMGCL",
"gene_synonyms": "HL",
"gene_synonyms_links": "",
"gene_name": "3-hydroxymethyl-3-methylglutaryl-CoA lyase",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000117305",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion matrix; Peroxisome",
"reaction": "(3S)-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA",
"targ_desciption": "Mitochondrial 3-hydroxymethyl-3-methylglutaryl-CoA lyase thatcatalyzes a cation-dependent cleavage of (S)-3-hydroxy-3-methylglutaryl-CoA into acetyl-CoA and acetoacetate, a key step inketogenesis. Terminal step in leucine catabolism. Ketone bodies (beta-hydroxybutyrate, acetoacetate and acetone) are essential as analternative source of energy to glucose, as lipid precursors and asregulators of metabolism. {ECO:0000269|PubMed:22847177,ECO:0000269|PubMed:22865860, ECO:0000269|PubMed:8566388}.",
"references": []
},
{
"targ_id": "T047",
"parent_targ_id": "T",
"full_name": "Acetyl-CoA carboxylase 2",
"abbrev": "ACC-beta",
"protein_names": "Acetyl-CoA carboxylase 2 (EC 6.4.1.2) (ACC-beta)",
"related_func_ids": "F02060103; F02060102",
"category": "enzyme",
"subcategories": "Allosteric enzyme, Ligase, Multifunctional enzyme",
"Uniport_ID": "O00763",
"Uniprot_name": "ACACB_HUMAN",
"EC_numbers": "6.4.1.2",
"gene_symbol": "ACACB",
"gene_synonyms": "ACC2, ACCB, HACC275",
"gene_synonyms_links": "",
"gene_name": "Acetyl-CoA carboxylase beta",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000076555",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion",
"reaction": "acetyl-CoA + ATP + hydrogencarbonate = ADP + H(+) + malonyl-CoA + phosphate",
"targ_desciption": "Mitochondrial enzyme that catalyzes the carboxylation ofacetyl-CoA to malonyl-CoA and plays a central role in fatty acidmetabolism (PubMed:16854592, PubMed:19236960, PubMed:20457939,PubMed:20952656, PubMed:19900410, PubMed:26976583). Catalyzes a 2 stepsreaction starting with the ATP-dependent carboxylation of the biotincarried by the biotin carboxyl carrier (BCC) domain followed by thetransfer of the carboxyl group from carboxylated biotin to acetyl-CoA(PubMed:19236960, PubMed:20457939, PubMed:20952656, PubMed:26976583).Through the production of malonyl-CoA that allosterically inhibitscarnitine palmitoyltransferase 1 at the mitochondria, negativelyregulates fatty acid oxidation (By similarity). Together with itscytosolic isozyme ACACA, which is involved in de novo fatty acidbiosynthesis, promotes lipid storage (By similarity).{ECO:0000250|UniProtKB:E9Q4Z2, ECO:0000269|PubMed:16854592,ECO:0000269|PubMed:19236960, ECO:0000269|PubMed:19900410,ECO:0000269|PubMed:20457939, ECO:0000269|PubMed:20952656,ECO:0000269|PubMed:26976583}.",
"references": [
"RC03781"
]
},
{
"targ_id": "T048",
"parent_targ_id": "T",
"full_name": "Cholesterol side-chain cleavage enzyme, mitochondrial",
"abbrev": "CYPXIA1",
"protein_names": "Cholesterol side-chain cleavage enzyme, mitochondrial (EC 1.14.15.6) (CYPXIA1) (Cholesterol desmolase) (Cytochrome P450 11A1) (Cytochrome P450(scc))",
"related_func_ids": "F020604",
"category": "enzyme",
"subcategories": "Monooxygenase, Oxidoreductase",
"Uniport_ID": "P05108",
"Uniprot_name": "CP11A_HUMAN",
"EC_numbers": "1.14.15.6",
"gene_symbol": "CYP11A1",
"gene_synonyms": "CYP11A, P450SCC",
"gene_synonyms_links": "",
"gene_name": "Cytochrome P450 family 11 subfamily A member 1",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000140459",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Peripheral membrane protein",
"reaction": "cholesterol + 6 H(+) + 3 O2 + 6 reduced [adrenodoxin] = 4-methylpentanal + 4 H2O + 6 oxidized [adrenodoxin] + pregnenolone; cholesterol + 2 H(+) + O2 + 2 reduced [adrenodoxin] = 22R-hydroxycholesterol + H2O + 2 oxidized [adrenodoxin]; 22R-hydroxycholesterol + 2 H(+) + O2 + 2 reduced [adrenodoxin]= (20R,22R)-20,22-dihydroxycholesterol + H2O + 2 oxidized[adrenodoxin]; (20R,22R)-20,22-dihydroxycholesterol + 2 H(+) + O2 + 2 reduced[adrenodoxin] = 4-methylpentanal + 2 H2O + 2 oxidized [adrenodoxin] +pregnenolone",
"targ_desciption": "A cytochrome P450 monooxygenase that catalyzes the side-chainhydroxylation and cleavage of cholesterol to pregnenolone, theprecursor of most steroid hormones (PubMed:21636783). Catalyzes threesequential oxidation reactions of cholesterol, namely the hydroxylationat C22 followed with the hydroxylation at C20 to yield 20R,22R-hydroxycholesterol that is further cleaved between C20 and C22 to yieldthe C21-steroid pregnenolone and 4-methylpentanal (PubMed:21636783).Mechanistically, uses molecular oxygen inserting one oxygen atom into asubstrate and reducing the second into a water molecule. Two electronsare provided by NADPH via a two-protein mitochondrial transfer systemcomprising flavoprotein FDXR (adrenodoxin/ferredoxin reductase) andnonheme iron-sulfur protein FDX1 or FDX2 (adrenodoxin/ferredoxin)(PubMed:21636783). {ECO:0000269|PubMed:21636783}.",
"references": []
},
{
"targ_id": "T049",
"parent_targ_id": "T",
"full_name": "Enoyl-CoA delta isomerase 2",
"abbrev": "DRS-1",
"protein_names": "Enoyl-CoA delta isomerase 2 (EC 5.3.3.8) (DRS-1) (Delta(3),delta(2)-enoyl-CoA isomerase) (D3,D2-enoyl-CoA isomerase) (Diazepam-binding inhibitor-related protein 1) (DBI-related protein 1) (Dodecenoyl-CoA isomerase) (Hepatocellular carcinoma-associated antigen 88) (Peroxisomal 3,2-trans-enoyl-CoA isomerase) (pECI) (Renal carcinoma antigen NY-REN-1)",
"related_func_ids": "F02060101",
"category": "enzyme",
"subcategories": "Isomerase",
"Uniport_ID": "O75521",
"Uniprot_name": "ECI2_HUMAN",
"EC_numbers": "5.3.3.8",
"gene_symbol": "ECI2",
"gene_synonyms": "ACBD2, DRS1, HCA88, PECI",
"gene_synonyms_links": "",
"gene_name": "Enoyl-CoA delta isomerase 2",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000198721",
"omim_id": "",
"genecard_link": "",
"locs": "[Isoform 1]: Mitochondrion; [Isoform 2]: Peroxisome matrix",
"reaction": "a (3Z)-enoyl-CoA = a 4-saturated (2E)-enoyl-CoA; (3Z)-octenoyl-CoA = (2E)-octenoyl-CoA; a (3E)-enoyl-CoA = a 4-saturated (2E)-enoyl-CoA; (2E)-tetradecenoyl-CoA = (3Z)-tetradecenoyl-CoA; (3E)-tetradecenoyl-CoA = (2E)-tetradecenoyl-CoA; (3E)-octenoyl-CoA = (2E)-octenoyl-CoA; (3E)-nonenoyl-CoA = (2E)-nonenoyl-CoA",
"targ_desciption": "Able to isomerize both 3-cis and 3-trans double bonds intothe 2-trans form in a range of enoyl-CoA species. Has a preference for3-trans substrates. {ECO:0000269|PubMed:10419495}.",
"references": []
},
{
"targ_id": "T050",
"parent_targ_id": "T",
"full_name": "Cytochrome P450 11B1, mitochondrial",
"abbrev": "CYPXIB1",
"protein_names": "Cytochrome P450 11B1, mitochondrial (CYPXIB1) (Cytochrome P-450c11) (Cytochrome P450C11) (Steroid 11-beta-hydroxylase, CYP11B1) (EC 1.14.15.4)",
"related_func_ids": "F02060101; F020604",
"category": "enzyme",
"subcategories": "Monooxygenase, Oxidoreductase",
"Uniport_ID": "P15538",
"Uniprot_name": "C11B1_HUMAN",
"EC_numbers": "1.14.15.4",
"gene_symbol": "CYP11B1",
"gene_synonyms": "CPN1, CYP11B, FHI, P450C11",
"gene_synonyms_links": "",
"gene_name": "Cytochrome P450 family 11 subfamily B member 1",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000160882",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Peripheral membrane protein",
"reaction": "a steroid + 2 H(+) + O2 + 2 reduced [adrenodoxin] = an 11beta-hydroxysteroid + H2O + 2 oxidized [adrenodoxin]; 11-deoxycortisol + 2 H(+) + O2 + 2 reduced [adrenodoxin] =cortisol + H2O + 2 oxidized [adrenodoxin]; 21-hydroxyprogesterone + 2 H(+) + O2 + 2 reduced [adrenodoxin]= corticosterone + H2O + 2 oxidized [adrenodoxin]",
"targ_desciption": "A cytochrome P450 monooxygenase involved in the biosynthesisof adrenal corticoids (PubMed:18215163). Catalyzes the hydroxylation ofcarbon hydrogen bond at 11-beta position of 11-deoxycortisol and 11-deoxycorticosterone/21-hydroxyprogesterone yielding cortisol orcorticosterone, respectively (PubMed:18215163). Mechanistically, usesmolecular oxygen inserting one oxygen atom into a substrate andreducing the second into a water molecule. Two electrons are providedby NADPH via a two-protein mitochondrial transfer system comprisingflavoprotein FDXR (adrenodoxin/ferredoxin reductase) and nonheme iron-sulfur protein FDX1 or FDX2 (adrenodoxin/ferredoxin) (PubMed:18215163).{ECO:0000269|PubMed:18215163}.",
"references": []
},
{
"targ_id": "T051",
"parent_targ_id": "T",
"full_name": "Malonyl-CoA-acyl carrier protein transacylase, mitochondrial",
"abbrev": "MCT",
"protein_names": "Malonyl-CoA-acyl carrier protein transacylase, mitochondrial (MCT) (EC 2.3.1.39) (Mitochondrial malonyl CoA:ACP acyltransferase) (Mitochondrial malonyltransferase) ([Acyl-carrier-protein] malonyltransferase)",
"related_func_ids": "F02060101",
"category": "enzyme",
"subcategories": "Transferase",
"Uniport_ID": "Q8IVS2",
"Uniprot_name": "FABD_HUMAN",
"EC_numbers": "2.3.1.39",
"gene_symbol": "MCAT",
"gene_synonyms": "fabD, FASN2C, MCT, MCT1, MT, NET62",
"gene_synonyms_links": "",
"gene_name": "Malonyl-CoA-acyl carrier protein transacylase",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000100294",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion",
"reaction": "holo-[ACP] + malonyl-CoA = CoA + malonyl-[ACP]",
"targ_desciption": "Catalyzes the transfer of a malonyl moiety from malonyl-CoAto the free thiol group of the phosphopantetheine arm of themitochondrial ACP protein (NDUFAB1). This suggests the existence of thebiosynthesis of fatty acids in mitochondria.{ECO:0000269|PubMed:12882974}.",
"references": []
},
{
"targ_id": "T052",
"parent_targ_id": "T",
"full_name": "Enoyl-CoA delta isomerase 1, mitochondrial",
"abbrev": "3,2-trans-enoyl-CoA isomerase",
"protein_names": "Enoyl-CoA delta isomerase 1, mitochondrial (EC 5.3.3.8) (3,2-trans-enoyl-CoA isomerase) (Delta(3),Delta(2)-enoyl-CoA isomerase) (D3,D2-enoyl-CoA isomerase) (Dodecenoyl-CoA isomerase)",
"related_func_ids": "F02060101",
"category": "enzyme",
"subcategories": "Isomerase",
"Uniport_ID": "P42126",
"Uniprot_name": "ECI1_HUMAN",
"EC_numbers": "5.3.3.8",
"gene_symbol": "ECI1",
"gene_synonyms": "DCI",
"gene_synonyms_links": "",
"gene_name": "Enoyl-CoA delta isomerase 1",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000167969",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion matrix",
"reaction": "a (3Z)-enoyl-CoA = a 4-saturated (2E)-enoyl-CoA; a (3E)-enoyl-CoA = a 4-saturated (2E)-enoyl-CoA; (3Z)-octenoyl-CoA = (2E)-octenoyl-CoA; (2E)-tetradecenoyl-CoA = (3Z)-tetradecenoyl-CoA",
"targ_desciption": "Able to isomerize both 3-cis and 3-trans double bonds intothe 2-trans form in a range of enoyl-CoA species.{ECO:0000269|PubMed:7818490}.",
"references": []
},
{
"targ_id": "T053",
"parent_targ_id": "T",
"full_name": "Glutaryl-CoA dehydrogenase, mitochondrial",
"abbrev": "GCD",
"protein_names": "Glutaryl-CoA dehydrogenase, mitochondrial (GCD) (EC 1.3.8.6)",
"related_func_ids": "F02060101; F02060102",
"category": "enzyme",
"subcategories": "Oxidoreductase",
"Uniport_ID": "Q92947",
"Uniprot_name": "GCDH_HUMAN",
"EC_numbers": "1.3.8.6",
"gene_symbol": "GCDH",
"gene_synonyms": "ACAD5",
"gene_synonyms_links": "",
"gene_name": "Glutaryl-CoA dehydrogenase",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000105607",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion matrix",
"reaction": "glutaryl-CoA + 2 H(+) + oxidized [electron-transferflavoprotein] = (2E)-butenoyl-CoA + CO2 + reduced [electron-transferflavoprotein]",
"targ_desciption": "Catalyzes the oxidative decarboxylation of glutaryl-CoA tocrotonyl-CoA and CO(2) in the degradative pathway of L-lysine, L-hydroxylysine, and L-tryptophan metabolism. It uses electron transferflavoprotein as its electron acceptor. Isoform Short is inactive.{ECO:0000269|PubMed:17176108, ECO:0000269|PubMed:6423663,ECO:0000269|PubMed:8541831}.",
"references": []
},
{
"targ_id": "T054",
"parent_targ_id": "T",
"full_name": "2,4-dienoyl-CoA reductase, mitochondrial",
"abbrev": "2,4-dienoyl-CoA reductase [NADPH]",
"protein_names": "2,4-dienoyl-CoA reductase, mitochondrial (EC 1.3.1.34) (2,4-dienoyl-CoA reductase [NADPH]) (4-enoyl-CoA reductase [NADPH]) (Short chain dehydrogenase/reductase family 18C member 1)",
"related_func_ids": "F02060101",
"category": "enzyme",
"subcategories": "Oxidoreductase",
"Uniport_ID": "Q16698",
"Uniprot_name": "DECR_HUMAN",
"EC_numbers": "1.3.1.34",
"gene_symbol": "DECR1",
"gene_synonyms": "DECR, SDR18C1",
"gene_synonyms_links": "",
"gene_name": "2,4-dienoyl-CoA reductase 1",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000104325",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion",
"reaction": "a 4,5-saturated-(2E)-enoyl-CoA + NADP(+) = a (2E,4E)-dienoyl-CoA + H(+) + NADPH; (2E,4E)-hexadienoyl-CoA + H(+) + NADPH = (3E)-hexenoyl-CoA +NADP(+)",
"targ_desciption": "Auxiliary enzyme of beta-oxidation. It participates in themetabolism of unsaturated fatty enoyl-CoA esters having double bonds inboth even- and odd-numbered positions in mitochondria. Catalyzes theNADP-dependent reduction of 2,4-dienoyl-CoA to yield trans-3-enoyl-CoA.{ECO:0000269|PubMed:15531764}.",
"references": []
},
{
"targ_id": "T055",
"parent_targ_id": "T",
"full_name": "D-beta-hydroxybutyrate dehydrogenase, mitochondrial",
"abbrev": "BDH",
"protein_names": "D-beta-hydroxybutyrate dehydrogenase, mitochondrial (EC 1.1.1.30) (3-hydroxybutyrate dehydrogenase) (BDH) (Short chain dehydrogenase/reductase family 9C member 1)",
"related_func_ids": "F02060201",
"category": "enzyme",
"subcategories": "Allosteric enzyme, Oxidoreductase",
"Uniport_ID": "Q02338",
"Uniprot_name": "BDH_HUMAN",
"EC_numbers": "1.1.1.30",
"gene_symbol": "BDH1",
"gene_synonyms": "BDH, SDR9C1",
"gene_synonyms_links": "",
"gene_name": "3-hydroxybutyrate dehydrogenase 1",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000161267",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Mitochondrion matrix",
"reaction": "(R)-3-hydroxybutanoate + NAD(+) = acetoacetate + H(+) + NADH",
"targ_desciption": "",
"references": []
},
{
"targ_id": "T056",
"parent_targ_id": "T",
"full_name": "Isovaleryl-CoA dehydrogenase, mitochondrial",
"abbrev": "IVD",
"protein_names": "Isovaleryl-CoA dehydrogenase, mitochondrial (IVD) (EC 1.3.8.4) (Butyryl-CoA dehydrogenase) (EC 1.3.8.1)",
"related_func_ids": "F02060101",
"category": "enzyme",
"subcategories": "Oxidoreductase",
"Uniport_ID": "P26440",
"Uniprot_name": "IVD_HUMAN",
"EC_numbers": "1.3.8.4; 1.3.8.1",
"gene_symbol": "IVD",
"gene_synonyms": "ACAD2",
"gene_synonyms_links": "",
"gene_name": "Isovaleryl-CoA dehydrogenase",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000128928",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion matrix",
"reaction": "3-methylbutanoyl-CoA + H(+) + oxidized [electron-transferflavoprotein] = 3-methylbut-2-enoyl-CoA + reduced [electron-transferflavoprotein]; H(+) + oxidized [electron-transfer flavoprotein] + pentanoyl-CoA = (2E)-pentenoyl-CoA + reduced [electron-transfer flavoprotein]; H(+) + hexanoyl-CoA + oxidized [electron-transferflavoprotein] = (2E)-hexenoyl-CoA + reduced [electron-transferflavoprotein]; butanoyl-CoA + H(+) + oxidized [electron-transferflavoprotein] = (2E)-butenoyl-CoA + reduced [electron-transferflavoprotein]",
"targ_desciption": "Catalyzes the conversion of isovaleryl-CoA/3-methylbutanoyl-CoA to 3-methylbut-2-enoyl-CoA as an intermediate step in the leucine(Leu) catabolic pathway (PubMed:7640268). To a lesser extent, is alsoable to catalyze the oxidation of other saturated short-chain acyl-CoAthioesters as pentanoyl-CoA, hexenoyl-CoA and butenoyl-CoA(PubMed:7640268). {ECO:0000269|PubMed:7640268}.",
"references": [
"RC01247"
]
},
{
"targ_id": "T057",
"parent_targ_id": "T",
"full_name": "Acetyl-CoA acetyltransferase, mitochondrial",
"abbrev": "T2",
"protein_names": "Acetyl-CoA acetyltransferase, mitochondrial (EC 2.3.1.9) (Acetoacetyl-CoA thiolase) (T2)",
"related_func_ids": "F02060101; F02060201; F02060202",
"category": "enzyme",
"subcategories": "Acyltransferase, Transferase",
"Uniport_ID": "P24752",
"Uniprot_name": "THIL_HUMAN",
"EC_numbers": "2.3.1.9",
"gene_symbol": "ACAT1",
"gene_synonyms": "ACAT, THIL",
"gene_synonyms_links": "",
"gene_name": "Acetyl-CoA acetyltransferase 1",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000075239",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion",
"reaction": "2 acetyl-CoA = acetoacetyl-CoA + CoA; acetyl-CoA + propanoyl-CoA = 2-methyl-3-oxobutanoyl-CoA + CoA",
"targ_desciption": "This is one of the enzymes that catalyzes the last step ofthe mitochondrial beta-oxidation pathway, an aerobic process breakingdown fatty acids into acetyl-CoA (PubMed:1715688, PubMed:7728148,PubMed:9744475). Using free coenzyme A/CoA, catalyzes the thiolyticcleavage of medium- to long-chain 3-oxoacyl-CoAs into acetyl-CoA and afatty acyl-CoA shortened by two carbon atoms (PubMed:1715688,PubMed:7728148, PubMed:9744475). The activity of the enzyme isreversible and it can also catalyze the condensation of two acetyl-CoAmolecules into acetoacetyl-CoA (PubMed:17371050). Thereby, it plays amajor role in ketone body metabolism (PubMed:17371050, PubMed:1715688,PubMed:7728148, PubMed:9744475). {ECO:0000269|PubMed:1715688,ECO:0000269|PubMed:17371050, ECO:0000269|PubMed:7728148,ECO:0000269|PubMed:9744475}.",
"references": []
}
]
}
