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{
"targ_id": "T001",
"parent_targ_id": "T",
"full_name": "NADH:ubiquinone reductase",
"abbrev": "Complex I",
"protein_names": "",
"related_func_ids": "F020103",
"category": "enzyme",
"subcategories": "",
"Uniport_ID": "",
"Uniprot_name": "",
"EC_numbers": "7.1.1.2",
"gene_symbol": "",
"gene_synonyms": "",
"gene_synonyms_links": "",
"gene_name": "",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "",
"omim_id": "",
"genecard_link": "",
"locs": "",
"reaction": "a ubiquinone + 5 H(+)(in) + NADH = a ubiquinol + 4 H(+)(out) +NAD(+)",
"targ_desciption": "",
"references": [
"RC00112",
"RC00117",
"RC00122",
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"RC00132",
"RC00137",
"RC00142",
"RC00147",
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"RC04459",
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"RC04486",
"RC04487",
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"RC04492",
"RC04494",
"RC04495",
"RC04500",
"RC04503",
"RC04507",
"RC04509",
"RC04510",
"RC04512",
"RC04515",
"RC04733",
"RC04759",
"RC04760",
"RC04913",
"RC04924",
"RC04932",
"RC04942",
"RC04954",
"RC04958"
]
},
{
"targ_id": "T001001",
"parent_targ_id": "T001",
"full_name": "NADH-ubiquinone oxidoreductase chain 1",
"abbrev": "Complex I subunit 1",
"protein_names": "NADH-ubiquinone oxidoreductase chain 1 (EC 7.1.1.2) (NADH dehydrogenase subunit 1)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "Translocase",
"Uniport_ID": "P03886",
"Uniprot_name": "NU1M_HUMAN",
"EC_numbers": "7.1.1.2",
"gene_symbol": "MT-ND1",
"gene_synonyms": "MTND1, NAD1, ND1",
"gene_synonyms_links": "",
"gene_name": "Mitochondrially encoded NADH:ubiquinone oxidoreductase core subunit 1",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000198888",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Multi-pass membrane protein",
"reaction": "a ubiquinone + 5 H(+)(in) + NADH = a ubiquinol + 4 H(+)(out) +NAD(+)",
"targ_desciption": "Core subunit of the mitochondrial membrane respiratory chainNADH dehydrogenase (Complex I) that is believed to belong to theminimal assembly required for catalysis. Complex I functions in thetransfer of electrons from NADH to the respiratory chain. The immediateelectron acceptor for the enzyme is believed to be ubiquinone (Bysimilarity). {ECO:0000250}.",
"references": []
},
{
"targ_id": "T001002",
"parent_targ_id": "T001",
"full_name": "NADH-ubiquinone oxidoreductase chain 2",
"abbrev": "Complex I subunit 2",
"protein_names": "NADH-ubiquinone oxidoreductase chain 2 (EC 7.1.1.2) (NADH dehydrogenase subunit 2)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "Translocase",
"Uniport_ID": "P03891",
"Uniprot_name": "NU2M_HUMAN",
"EC_numbers": "7.1.1.2",
"gene_symbol": "MT-ND2",
"gene_synonyms": "MTND2, NAD2, ND2",
"gene_synonyms_links": "",
"gene_name": "Mitochondrially encoded NADH:ubiquinone oxidoreductase core subunit 2",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000198763",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Multi-pass membrane protein",
"reaction": "a ubiquinone + 5 H(+)(in) + NADH = a ubiquinol + 4 H(+)(out) +NAD(+)",
"targ_desciption": "Core subunit of the mitochondrial membrane respiratory chainNADH dehydrogenase (Complex I) that is believed to belong to theminimal assembly required for catalysis. Complex I functions in thetransfer of electrons from NADH to the respiratory chain. The immediateelectron acceptor for the enzyme is believed to be ubiquinone (Bysimilarity). {ECO:0000250}.",
"references": []
},
{
"targ_id": "T001003",
"parent_targ_id": "T001",
"full_name": "NADH-ubiquinone oxidoreductase chain 3",
"abbrev": "Complex I subunit 3",
"protein_names": "NADH-ubiquinone oxidoreductase chain 3 (EC 7.1.1.2) (NADH dehydrogenase subunit 3)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "Translocase",
"Uniport_ID": "P03897",
"Uniprot_name": "NU3M_HUMAN",
"EC_numbers": "7.1.1.2",
"gene_symbol": "MT-ND3",
"gene_synonyms": "MTND3, NAD3, ND3",
"gene_synonyms_links": "",
"gene_name": "Mitochondrially encoded NADH:ubiquinone oxidoreductase core subunit 3",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000198840",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion membrane; Multi-pass membrane protein",
"reaction": "a ubiquinone + 5 H(+)(in) + NADH = a ubiquinol + 4 H(+)(out) +NAD(+)",
"targ_desciption": "Core subunit of the mitochondrial membrane respiratory chainNADH dehydrogenase (Complex I) that is believed to belong to theminimal assembly required for catalysis. Complex I functions in thetransfer of electrons from NADH to the respiratory chain. The immediateelectron acceptor for the enzyme is believed to be ubiquinone (Bysimilarity). {ECO:0000250}.",
"references": []
},
{
"targ_id": "T001004",
"parent_targ_id": "T001",
"full_name": "NADH-ubiquinone oxidoreductase chain 4",
"abbrev": "Complex I subunit 4",
"protein_names": "NADH-ubiquinone oxidoreductase chain 4 (EC 7.1.1.2) (NADH dehydrogenase subunit 4)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "Translocase",
"Uniport_ID": "P03905",
"Uniprot_name": "NU4M_HUMAN",
"EC_numbers": "7.1.1.2",
"gene_symbol": "MT-ND4",
"gene_synonyms": "LHON, MTND4, NAD4, ND4",
"gene_synonyms_links": "",
"gene_name": "Mitochondrially encoded NADH:ubiquinone oxidoreductase core subunit 4",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000198886",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion membrane; Multi-pass membrane protein",
"reaction": "a ubiquinone + 5 H(+)(in) + NADH = a ubiquinol + 4 H(+)(out) +NAD(+)",
"targ_desciption": "Core subunit of the mitochondrial membrane respiratory chainNADH dehydrogenase (Complex I) that is believed to belong to theminimal assembly required for catalysis. Complex I functions in thetransfer of electrons from NADH to the respiratory chain. The immediateelectron acceptor for the enzyme is believed to be ubiquinone (Bysimilarity). {ECO:0000250}.",
"references": []
},
{
"targ_id": "T001005",
"parent_targ_id": "T001",
"full_name": "NADH-ubiquinone oxidoreductase chain 4L",
"abbrev": "Complex I subunit 4L",
"protein_names": "NADH-ubiquinone oxidoreductase chain 4L (EC 7.1.1.2) (NADH dehydrogenase subunit 4L)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "Translocase",
"Uniport_ID": "P03901",
"Uniprot_name": "NU4LM_HUMAN",
"EC_numbers": "7.1.1.2",
"gene_symbol": "MT-ND4L",
"gene_synonyms": "MTND4L, NAD4L, ND4L",
"gene_synonyms_links": "",
"gene_name": "Mitochondrially encoded NADH:ubiquinone oxidoreductase core subunit 4L",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000212907",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion membrane; Multi-pass membrane protein",
"reaction": "a ubiquinone + 5 H(+)(in) + NADH = a ubiquinol + 4 H(+)(out) +NAD(+)",
"targ_desciption": "Core subunit of the mitochondrial membrane respiratory chainNADH dehydrogenase (Complex I) that is believed to belong to theminimal assembly required for catalysis. Complex I functions in thetransfer of electrons from NADH to the respiratory chain. The immediateelectron acceptor for the enzyme is believed to be ubiquinone (Bysimilarity). {ECO:0000250}.",
"references": []
},
{
"targ_id": "T001006",
"parent_targ_id": "T001",
"full_name": "NADH-ubiquinone oxidoreductase chain 5",
"abbrev": "Complex I subunit 5",
"protein_names": "NADH-ubiquinone oxidoreductase chain 5 (EC 7.1.1.2) (NADH dehydrogenase subunit 5)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "Translocase",
"Uniport_ID": "P03915",
"Uniprot_name": "NU5M_HUMAN",
"EC_numbers": "7.1.1.2",
"gene_symbol": "MT-ND5",
"gene_synonyms": "MTND5, NAD5, ND5",
"gene_synonyms_links": "",
"gene_name": "Mitochondrially encoded NADH:ubiquinone oxidoreductase core subunit 5",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000198786",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Multi-pass membrane protein",
"reaction": "a ubiquinone + 5 H(+)(in) + NADH = a ubiquinol + 4 H(+)(out) +NAD(+)",
"targ_desciption": "Core subunit of the mitochondrial membrane respiratory chainNADH dehydrogenase (Complex I) that is believed to belong to theminimal assembly required for catalysis. Complex I functions in thetransfer of electrons from NADH to the respiratory chain. The immediateelectron acceptor for the enzyme is believed to be ubiquinone (Bysimilarity). {ECO:0000250}.",
"references": []
},
{
"targ_id": "T001007",
"parent_targ_id": "T001",
"full_name": "NADH-ubiquinone oxidoreductase chain 6",
"abbrev": "Complex I subunit 6",
"protein_names": "NADH-ubiquinone oxidoreductase chain 6 (EC 7.1.1.2) (NADH dehydrogenase subunit 6)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "Translocase",
"Uniport_ID": "P03923",
"Uniprot_name": "NU6M_HUMAN",
"EC_numbers": "7.1.1.2",
"gene_symbol": "MT-ND6",
"gene_synonyms": "MTND6, NAD6, ND6",
"gene_synonyms_links": "",
"gene_name": "Mitochondrially encoded NADH:ubiquinone oxidoreductase core subunit 6",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000198695",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion membrane; Multi-pass membrane protein",
"reaction": "a ubiquinone + 5 H(+)(in) + NADH = a ubiquinol + 4 H(+)(out) +NAD(+)",
"targ_desciption": "Core subunit of the mitochondrial membrane respiratory chainNADH dehydrogenase (Complex I) that is believed to belong to theminimal assembly required for catalysis. Complex I functions in thetransfer of electrons from NADH to the respiratory chain. The immediateelectron acceptor for the enzyme is believed to be ubiquinone (Bysimilarity). {ECO:0000250}.",
"references": []
},
{
"targ_id": "T001008",
"parent_targ_id": "T001",
"full_name": "NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1",
"abbrev": "CI-MWFE",
"protein_names": "NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 (Complex I-MWFE) (CI-MWFE) (NADH-ubiquinone oxidoreductase MWFE subunit)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "O15239",
"Uniprot_name": "NDUA1_HUMAN",
"EC_numbers": "",
"gene_symbol": "NDUFA1",
"gene_synonyms": "CI-MWFE, MWFE",
"gene_synonyms_links": "",
"gene_name": "NADH:ubiquinone oxidoreductase subunit A1",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000125356",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Single-pass membrane protein; Matrix side",
"reaction": "",
"targ_desciption": "Accessory subunit of the mitochondrial membrane respiratorychain NADH dehydrogenase (Complex I), that is believed not to beinvolved in catalysis. Complex I functions in the transfer of electronsfrom NADH to the respiratory chain. The immediate electron acceptor forthe enzyme is believed to be ubiquinone. {ECO:0000269|PubMed:27626371}.",
"references": []
},
{
"targ_id": "T001009",
"parent_targ_id": "T001",
"full_name": "NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2",
"abbrev": "CI-B8",
"protein_names": "NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 (Complex I-B8) (CI-B8) (NADH-ubiquinone oxidoreductase B8 subunit)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "O43678",
"Uniprot_name": "NDUA2_HUMAN",
"EC_numbers": "",
"gene_symbol": "NDUFA2",
"gene_synonyms": "B8",
"gene_synonyms_links": "",
"gene_name": "NADH:ubiquinone oxidoreductase subunit A2",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000131495",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Peripheral membrane protein; Matrix side",
"reaction": "",
"targ_desciption": "Accessory subunit of the mitochondrial membrane respiratorychain NADH dehydrogenase (Complex I), that is believed not to beinvolved in catalysis. Complex I functions in the transfer of electronsfrom NADH to the respiratory chain. The immediate electron acceptor forthe enzyme is believed to be ubiquinone. {ECO:0000269|PubMed:27626371}.",
"references": []
},
{
"targ_id": "T001010",
"parent_targ_id": "T001",
"full_name": "NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6",
"abbrev": "CI-B14",
"protein_names": "NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 (Complex I-B14) (CI-B14) (LYR motif-containing protein 6) (NADH-ubiquinone oxidoreductase B14 subunit)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "P56556",
"Uniprot_name": "NDUA6_HUMAN",
"EC_numbers": "",
"gene_symbol": "NDUFA6",
"gene_synonyms": "B14, CI-B14, LYRM6, NADHB14",
"gene_synonyms_links": "",
"gene_name": "NADH:ubiquinone oxidoreductase subunit A6",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000184983",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Peripheral membrane protein; Matrix side",
"reaction": "",
"targ_desciption": "Accessory subunit of the mitochondrial membrane respiratorychain NADH dehydrogenase (Complex I), that is believed to be notinvolved in catalysis. Required for proper complex I assembly(PubMed:30245030). Complex I functions in the transfer of electronsfrom NADH to the respiratory chain. The immediate electron acceptor forthe enzyme is believed to be ubiquinone. {ECO:0000269|PubMed:27626371,ECO:0000269|PubMed:30245030}.",
"references": []
},
{
"targ_id": "T001011",
"parent_targ_id": "T001",
"full_name": "NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial",
"abbrev": "CI-39kD",
"protein_names": "NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial (Complex I-39kD) (CI-39kD) (NADH-ubiquinone oxidoreductase 39 kDa subunit)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "Q16795",
"Uniprot_name": "NDUA9_HUMAN",
"EC_numbers": "",
"gene_symbol": "NDUFA9",
"gene_synonyms": "CI-39k, NDUFS2L, SDR22E1",
"gene_synonyms_links": "",
"gene_name": "NADH:ubiquinone oxidoreductase subunit A9",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000139180",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion matrix",
"reaction": "",
"targ_desciption": "Accessory subunit of the mitochondrial membrane respiratorychain NADH dehydrogenase (Complex I), that is believed not to beinvolved in catalysis. Required for proper complex I assembly(PubMed:28671271). Complex I functions in the transfer of electronsfrom NADH to the respiratory chain. The immediate electron acceptor forthe enzyme is believed to be ubiquinone. {ECO:0000269|PubMed:22114105,ECO:0000269|PubMed:27626371, ECO:0000269|PubMed:28671271}.",
"references": []
},
{
"targ_id": "T001012",
"parent_targ_id": "T001",
"full_name": "NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial",
"abbrev": "CI-42kD",
"protein_names": "NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial (Complex I-42kD) (CI-42kD) (NADH-ubiquinone oxidoreductase 42 kDa subunit)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "O95299",
"Uniprot_name": "NDUAA_HUMAN",
"EC_numbers": "",
"gene_symbol": "NDUFA10",
"gene_synonyms": "CI-42k",
"gene_synonyms_links": "",
"gene_name": "NADH:ubiquinone oxidoreductase subunit A10",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000130414",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion matrix",
"reaction": "",
"targ_desciption": "Accessory subunit of the mitochondrial membrane respiratorychain NADH dehydrogenase (Complex I), that is believed not to beinvolved in catalysis. Complex I functions in the transfer of electronsfrom NADH to the respiratory chain. The immediate electron acceptor forthe enzyme is believed to be ubiquinone. {ECO:0000269|PubMed:27626371}.",
"references": []
},
{
"targ_id": "T001013",
"parent_targ_id": "T001",
"full_name": "NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11",
"abbrev": "CI-B14.7",
"protein_names": "NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 (Complex I-B14.7) (CI-B14.7) (NADH-ubiquinone oxidoreductase subunit B14.7)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "Q86Y39",
"Uniprot_name": "NDUAB_HUMAN",
"EC_numbers": "",
"gene_symbol": "NDUFA11",
"gene_synonyms": "B14.7",
"gene_synonyms_links": "",
"gene_name": "NADH:ubiquinone oxidoreductase subunit A11",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000174886",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Multi-pass membrane protein; Matrix side",
"reaction": "",
"targ_desciption": "Accessory subunit of the mitochondrial membrane respiratorychain NADH dehydrogenase (Complex I), that is believed not to beinvolved in catalysis. Complex I functions in the transfer of electronsfrom NADH to the respiratory chain. The immediate electron acceptor forthe enzyme is believed to be ubiquinone. {ECO:0000269|PubMed:27626371}.",
"references": []
},
{
"targ_id": "T001014",
"parent_targ_id": "T001",
"full_name": "NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12",
"abbrev": "Complex I-B17.2",
"protein_names": "NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 (13 kDa differentiation-associated protein) (Complex I-B17.2) (CI-B17.2) (CIB17.2) (NADH-ubiquinone oxidoreductase subunit B17.2)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "Q9UI09",
"Uniprot_name": "NDUAC_HUMAN",
"EC_numbers": "",
"gene_symbol": "NDUFA12",
"gene_synonyms": "B17.2, DAP13",
"gene_synonyms_links": "",
"gene_name": "NADH:ubiquinone oxidoreductase subunit A12",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000184752",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Peripheral membrane protein; Matrix side",
"reaction": "",
"targ_desciption": "Accessory subunit of the mitochondrial membrane respiratorychain NADH dehydrogenase (Complex I), that is believed not to beinvolved in catalysis. Complex I functions in the transfer of electronsfrom NADH to the respiratory chain. The immediate electron acceptor forthe enzyme is believed to be ubiquinone. {ECO:0000269|PubMed:27626371}.",
"references": []
},
{
"targ_id": "T001015",
"parent_targ_id": "T001",
"full_name": "NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13",
"abbrev": "Complex I-B16.6",
"protein_names": "NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 (Cell death regulatory protein GRIM-19) (Complex I-B16.6) (CI-B16.6) (Gene associated with retinoic and interferon-induced mortality 19 protein) (GRIM-19) (Gene associated with retinoic and IFN-induced mortality 19 protein) (NADH-ubiquinone oxidoreductase B16.6 subunit)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "Q9P0J0",
"Uniprot_name": "NDUAD_HUMAN",
"EC_numbers": "",
"gene_symbol": "NDUFA13",
"gene_synonyms": "B16.6, CDA016, CGI-39, GRIM-19, GRIM19",
"gene_synonyms_links": "",
"gene_name": "NADH:ubiquinone oxidoreductase subunit A13",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000186010",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Single-pass membrane protein; Matrix side; Nucleus",
"reaction": "",
"targ_desciption": "Accessory subunit of the mitochondrial membrane respiratorychain NADH dehydrogenase (Complex I), that is believed not to beinvolved in catalysis (PubMed:27626371). Complex I functions in thetransfer of electrons from NADH to the respiratory chain. The immediateelectron acceptor for the enzyme is believed to be ubiquinone(PubMed:27626371). Involved in the interferon/all-trans-retinoic acid(IFN/RA) induced cell death. This apoptotic activity is inhibited byinteraction with viral IRF1. Prevents the transactivation of STAT3target genes. May play a role in CARD15-mediated innate mucosalresponses and serve to regulate intestinal epithelial cell responses tomicrobes (PubMed:15753091). {ECO:0000269|PubMed:12628925,ECO:0000269|PubMed:12867595, ECO:0000269|PubMed:15753091,ECO:0000269|PubMed:27626371}.",
"references": []
},
{
"targ_id": "T001016",
"parent_targ_id": "T001",
"full_name": "NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3",
"abbrev": "CI-B12",
"protein_names": "NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 (Complex I-B12) (CI-B12) (NADH-ubiquinone oxidoreductase B12 subunit)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "O43676",
"Uniprot_name": "NDUB3_HUMAN",
"EC_numbers": "",
"gene_symbol": "NDUFB3",
"gene_synonyms": "B12",
"gene_synonyms_links": "",
"gene_name": "NADH:ubiquinone oxidoreductase subunit B3",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000119013",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Single-pass membrane protein; Matrix side",
"reaction": "",
"targ_desciption": "Accessory subunit of the mitochondrial membrane respiratorychain NADH dehydrogenase (Complex I), that is believed not to beinvolved in catalysis. Complex I functions in the transfer of electronsfrom NADH to the respiratory chain. The immediate electron acceptor forthe enzyme is believed to be ubiquinone. {ECO:0000269|PubMed:27626371}.",
"references": []
},
{
"targ_id": "T001017",
"parent_targ_id": "T001",
"full_name": "NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial",
"abbrev": "CI-ASHI",
"protein_names": "NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial (Complex I-ASHI) (CI-ASHI) (NADH-ubiquinone oxidoreductase ASHI subunit)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "O95169",
"Uniprot_name": "NDUB8_HUMAN",
"EC_numbers": "",
"gene_symbol": "NDUFB8",
"gene_synonyms": "ASHI, CI-ASHI",
"gene_synonyms_links": "",
"gene_name": "NADH:ubiquinone oxidoreductase subunit B8",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000166136",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Single-pass membrane protein; Matrix side",
"reaction": "",
"targ_desciption": "Accessory subunit of the mitochondrial membrane respiratorychain NADH dehydrogenase (Complex I), that is believed not to beinvolved in catalysis. Complex I functions in the transfer of electronsfrom NADH to the respiratory chain. The immediate electron acceptor forthe enzyme is believed to be ubiquinone. {ECO:0000269|PubMed:27626371}.",
"references": []
},
{
"targ_id": "T001018",
"parent_targ_id": "T001",
"full_name": "NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9",
"abbrev": "CI-B22",
"protein_names": "NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 (Complex I-B22) (CI-B22) (LYR motif-containing protein 3) (NADH-ubiquinone oxidoreductase B22 subunit)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "Q9Y6M9",
"Uniprot_name": "NDUB9_HUMAN",
"EC_numbers": "",
"gene_symbol": "NDUFB9",
"gene_synonyms": "B22, LYRM3, UQOR22",
"gene_synonyms_links": "",
"gene_name": "NADH:ubiquinone oxidoreductase subunit B9",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000147684",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Peripheral membrane protein; Matrix side",
"reaction": "",
"targ_desciption": "Accessory subunit of the mitochondrial membrane respiratorychain NADH dehydrogenase (Complex I), that is believed to be notinvolved in catalysis. Complex I functions in the transfer of electronsfrom NADH to the respiratory chain. The immediate electron acceptor forthe enzyme is believed to be ubiquinone. {ECO:0000269|PubMed:27626371}.",
"references": []
},
{
"targ_id": "T001019",
"parent_targ_id": "T001",
"full_name": "NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10",
"abbrev": "CI-PDSW",
"protein_names": "NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 (Complex I-PDSW) (CI-PDSW) (NADH-ubiquinone oxidoreductase PDSW subunit)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "O96000",
"Uniprot_name": "NDUBA_HUMAN",
"EC_numbers": "",
"gene_symbol": "NDUFB10",
"gene_synonyms": "PDSW",
"gene_synonyms_links": "",
"gene_name": "NADH:ubiquinone oxidoreductase subunit B10",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000140990",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Peripheral membrane protein; Matrix side",
"reaction": "",
"targ_desciption": "Accessory subunit of the mitochondrial membrane respiratorychain NADH dehydrogenase (Complex I), that is believed not to beinvolved in catalysis. Complex I functions in the transfer of electronsfrom NADH to the respiratory chain. The immediate electron acceptor forthe enzyme is believed to be ubiquinone. {ECO:0000269|PubMed:27626371}.",
"references": []
},
{
"targ_id": "T001020",
"parent_targ_id": "T001",
"full_name": "NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial",
"abbrev": "CI-ESSS",
"protein_names": "NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial (Complex I-ESSS) (CI-ESSS) (NADH-ubiquinone oxidoreductase ESSS subunit) (Neuronal protein 17.3) (Np17.3) (p17.3)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "Q9NX14",
"Uniprot_name": "NDUBB_HUMAN",
"EC_numbers": "",
"gene_symbol": "NDUFB11",
"gene_synonyms": "ESSS, Np15, NP17.3",
"gene_synonyms_links": "",
"gene_name": "NADH:ubiquinone oxidoreductase subunit B11",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000147123",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Single-pass membrane protein",
"reaction": "",
"targ_desciption": "Accessory subunit of the mitochondrial membrane respiratorychain NADH dehydrogenase (Complex I), that is believed not to beinvolved in catalysis. Complex I functions in the transfer of electronsfrom NADH to the respiratory chain. The immediate electron acceptor forthe enzyme is believed to be ubiquinone. {ECO:0000269|PubMed:27626371}.",
"references": []
},
{
"targ_id": "T001021",
"parent_targ_id": "T001",
"full_name": "NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial",
"abbrev": "CI-75kD",
"protein_names": "NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial (EC 7.1.1.2) (Complex I-75kD) (CI-75kD)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "Oxidoreductase, Translocase",
"Uniport_ID": "P28331",
"Uniprot_name": "NDUS1_HUMAN",
"EC_numbers": "7.1.1.2",
"gene_symbol": "NDUFS1",
"gene_synonyms": "CI-75k",
"gene_synonyms_links": "",
"gene_name": "NADH:ubiquinone oxidoreductase core subunit S1",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000023228",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane",
"reaction": "a ubiquinone + 5 H(+)(in) + NADH = a ubiquinol + 4 H(+)(out) +NAD(+)",
"targ_desciption": "Core subunit of the mitochondrial membrane respiratory chainNADH dehydrogenase (Complex I) that is believed to belong to theminimal assembly required for catalysis. Complex I functions in thetransfer of electrons from NADH to the respiratory chain. The immediateelectron acceptor for the enzyme is believed to be ubiquinone (Bysimilarity). This is the largest subunit of complex I and it is acomponent of the iron-sulfur (IP) fragment of the enzyme. It may formpart of the active site crevice where NADH is oxidized. {ECO:0000250}.",
"references": []
},
{
"targ_id": "T001022",
"parent_targ_id": "T001",
"full_name": "NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial",
"abbrev": "CI-49kD",
"protein_names": "NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial (EC 7.1.1.2) (Complex I-49kD) (CI-49kD) (NADH-ubiquinone oxidoreductase 49 kDa subunit)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "Oxidoreductase, Translocase",
"Uniport_ID": "O75306",
"Uniprot_name": "NDUS2_HUMAN",
"EC_numbers": "7.1.1.2",
"gene_symbol": "NDUFS2",
"gene_synonyms": "CI-49",
"gene_synonyms_links": "",
"gene_name": "NADH:ubiquinone oxidoreductase core subunit S2",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000158864",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Peripheral membrane protein; Matrix side",
"reaction": "a ubiquinone + 5 H(+)(in) + NADH = a ubiquinol + 4 H(+)(out) +NAD(+)",
"targ_desciption": "Core subunit of the mitochondrial membrane respiratory chainNADH dehydrogenase (Complex I) that is believed to belong to theminimal assembly required for catalysis. Complex I functions in thetransfer of electrons from NADH to the respiratory chain. The immediateelectron acceptor for the enzyme is believed to be ubiquinone(PubMed:12611891). {ECO:0000269|PubMed:12611891}.",
"references": []
},
{
"targ_id": "T001023",
"parent_targ_id": "T001",
"full_name": "NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial",
"abbrev": "CI-30kD",
"protein_names": "NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial (EC 7.1.1.2) (Complex I-30kD) (CI-30kD) (NADH-ubiquinone oxidoreductase 30 kDa subunit)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "Oxidoreductase, Translocase",
"Uniport_ID": "O75489",
"Uniprot_name": "NDUS3_HUMAN",
"EC_numbers": "7.1.1.2",
"gene_symbol": "NDUFS3",
"gene_synonyms": "CI-30",
"gene_synonyms_links": "",
"gene_name": "NADH:ubiquinone oxidoreductase core subunit S3",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000213619",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane",
"reaction": "a ubiquinone + 5 H(+)(in) + NADH = a ubiquinol + 4 H(+)(out) +NAD(+)",
"targ_desciption": "Core subunit of the mitochondrial membrane respiratory chainNADH dehydrogenase (Complex I) that is believed to belong to theminimal assembly required for catalysis. Complex I functions in thetransfer of electrons from NADH to the respiratory chain. The immediateelectron acceptor for the enzyme is believed to be ubiquinone (Bysimilarity). {ECO:0000250}.",
"references": []
},
{
"targ_id": "T001024",
"parent_targ_id": "T001",
"full_name": "NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial",
"abbrev": "CI-18 kDa",
"protein_names": "NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial (Complex I-18 kDa) (CI-18 kDa) (Complex I-AQDQ) (CI-AQDQ) (NADH-ubiquinone oxidoreductase 18 kDa subunit)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "O43181",
"Uniprot_name": "NDUS4_HUMAN",
"EC_numbers": "7.1.1.2",
"gene_symbol": "NDUFS4",
"gene_synonyms": "AQDQ, CI-18",
"gene_synonyms_links": "",
"gene_name": "NADH:ubiquinone oxidoreductase subunit S4",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000164258",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Peripheral membrane protein; Matrix side",
"reaction": "a ubiquinone + 5 H(+)(in) + NADH = a ubiquinol + 4 H(+)(out) +NAD(+)",
"targ_desciption": "Accessory subunit of the mitochondrial membrane respiratorychain NADH dehydrogenase (Complex I), that is believed not to beinvolved in catalysis. Complex I functions in the transfer of electronsfrom NADH to the respiratory chain. The immediate electron acceptor forthe enzyme is believed to be ubiquinone. {ECO:0000269|PubMed:12611891,ECO:0000269|PubMed:9463323}.",
"references": []
},
{
"targ_id": "T001025",
"parent_targ_id": "T001",
"full_name": "NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial",
"abbrev": "CI-13kD-A",
"protein_names": "NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial (Complex I-13kD-A) (CI-13kD-A) (NADH-ubiquinone oxidoreductase 13 kDa-A subunit)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "O75380",
"Uniprot_name": "NDUS6_HUMAN",
"EC_numbers": "7.1.1.2",
"gene_symbol": "NDUFS6",
"gene_synonyms": "CI-13kA",
"gene_synonyms_links": "",
"gene_name": "NADH:ubiquinone oxidoreductase subunit S6",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000145494",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Peripheral membrane protein; Matrix side",
"reaction": "a ubiquinone + 5 H(+)(in) + NADH = a ubiquinol + 4 H(+)(out) +NAD(+)",
"targ_desciption": "Accessory subunit of the mitochondrial membrane respiratorychain NADH dehydrogenase (Complex I), that is believed not to beinvolved in catalysis. Complex I functions in the transfer of electronsfrom NADH to the respiratory chain. The immediate electron acceptor forthe enzyme is believed to be ubiquinone. {ECO:0000269|PubMed:27626371}.",
"references": []
},
{
"targ_id": "T001026",
"parent_targ_id": "T001",
"full_name": "NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial",
"abbrev": "CI-20kD",
"protein_names": "NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial (EC 7.1.1.2) (Complex I-20kD) (CI-20kD) (NADH-ubiquinone oxidoreductase 20 kDa subunit) (PSST subunit)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "Oxidoreductase, Translocase",
"Uniport_ID": "O75251",
"Uniprot_name": "NDUS7_HUMAN",
"EC_numbers": "7.1.1.2",
"gene_symbol": "NDUFS7",
"gene_synonyms": "CI-20, FLJ45860, FLJ46880, PSST",
"gene_synonyms_links": "",
"gene_name": "NADH:ubiquinone oxidoreductase core subunit S7",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000115286",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion",
"reaction": "a ubiquinone + 5 H(+)(in) + NADH = a ubiquinol + 4 H(+)(out) +NAD(+)",
"targ_desciption": "Core subunit of the mitochondrial membrane respiratory chainNADH dehydrogenase (Complex I) that is believed to belong to theminimal assembly required for catalysis. Complex I functions in thetransfer of electrons from NADH to the respiratory chain. The immediateelectron acceptor for the enzyme is believed to be ubiquinone.{ECO:0000305|PubMed:12611891}.",
"references": []
},
{
"targ_id": "T001027",
"parent_targ_id": "T001",
"full_name": "NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial",
"abbrev": "CI-23kD",
"protein_names": "NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial (EC 7.1.1.2) (Complex I-23kD) (CI-23kD) (NADH-ubiquinone oxidoreductase 23 kDa subunit) (TYKY subunit)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "Oxidoreductase, Translocase",
"Uniport_ID": "O00217",
"Uniprot_name": "NDUS8_HUMAN",
"EC_numbers": "7.1.1.2",
"gene_symbol": "NDUFS8",
"gene_synonyms": "CI-23k, TYKY",
"gene_synonyms_links": "",
"gene_name": "NADH:ubiquinone oxidoreductase core subunit S8",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000110717",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion",
"reaction": "a ubiquinone + 5 H(+)(in) + NADH = a ubiquinol + 4 H(+)(out) +NAD(+)",
"targ_desciption": "Core subunit of the mitochondrial membrane respiratory chainNADH dehydrogenase (Complex I) that is believed to belong to theminimal assembly required for catalysis. Complex I functions in thetransfer of electrons from NADH to the respiratory chain. The immediateelectron acceptor for the enzyme is believed to be ubiquinone.{ECO:0000305|PubMed:12611891}.",
"references": []
},
{
"targ_id": "T001028",
"parent_targ_id": "T001",
"full_name": "NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial",
"abbrev": "CI-51kD",
"protein_names": "NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial (EC 7.1.1.2) (Complex I-51kD) (CI-51kD) (NADH dehydrogenase flavoprotein 1) (NADH-ubiquinone oxidoreductase 51 kDa subunit)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "Oxidoreductase, Translocase",
"Uniport_ID": "P49821",
"Uniprot_name": "NDUV1_HUMAN",
"EC_numbers": "7.1.1.2",
"gene_symbol": "NDUFV1",
"gene_synonyms": "CI-51K",
"gene_synonyms_links": "",
"gene_name": "NADH:ubiquinone oxidoreductase core subunit V1",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000167792",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Peripheral membrane protein; Matrix side",
"reaction": "a ubiquinone + 5 H(+)(in) + NADH = a ubiquinol + 4 H(+)(out) +NAD(+)",
"targ_desciption": "Core subunit of the mitochondrial membrane respiratory chainNADH dehydrogenase (Complex I) that is believed to belong to theminimal assembly required for catalysis. Complex I functions in thetransfer of electrons from NADH to the respiratory chain. The immediateelectron acceptor for the enzyme is believed to be ubiquinone (Bysimilarity). {ECO:0000250}.",
"references": []
},
{
"targ_id": "T001029",
"parent_targ_id": "T001",
"full_name": "NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial",
"abbrev": "",
"protein_names": "NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial (EC 7.1.1.2) (NADH-ubiquinone oxidoreductase 24 kDa subunit)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "Oxidoreductase, Translocase",
"Uniport_ID": "P19404",
"Uniprot_name": "NDUV2_HUMAN",
"EC_numbers": "7.1.1.2",
"gene_symbol": "NDUFV2",
"gene_synonyms": "CI-24k",
"gene_synonyms_links": "",
"gene_name": "NADH:ubiquinone oxidoreductase core subunit V2",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000178127",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane",
"reaction": "a ubiquinone + 5 H(+)(in) + NADH = a ubiquinol + 4 H(+)(out) +NAD(+)",
"targ_desciption": "Core subunit of the mitochondrial membrane respiratory chainNADH dehydrogenase (Complex I) that is believed to belong to theminimal assembly required for catalysis. Complex I functions in thetransfer of electrons from NADH to the respiratory chain. The immediateelectron acceptor for the enzyme is believed to be ubiquinone (Bysimilarity). {ECO:0000250}.",
"references": []
},
{
"targ_id": "T001030",
"parent_targ_id": "T001",
"full_name": "Complex I intermediate-associated protein 30, mitochondrial",
"abbrev": "",
"protein_names": "Complex I intermediate-associated protein 30, mitochondrial (NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 1)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "Chaperone",
"Uniport_ID": "Q9Y375",
"Uniprot_name": "CIA30_HUMAN",
"EC_numbers": "",
"gene_symbol": "NDUFAF1",
"gene_synonyms": "CGI-65, CIA30",
"gene_synonyms_links": "",
"gene_name": "NADH:ubiquinone oxidoreductase complex assembly factor 1",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000137806",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion; Mitochondrion matrix",
"reaction": "",
"targ_desciption": "Chaperone protein involved in early stages of the assembly ofmitochondrial NADH:ubiquinone oxidoreductase complex (complex I).{ECO:0000269|PubMed:16218961, ECO:0000269|PubMed:17557076}.",
"references": []
},
{
"targ_id": "T001031",
"parent_targ_id": "T001",
"full_name": "NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 2",
"abbrev": "Mimitin",
"protein_names": "NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 2 (B17.2-like) (B17.2L) (Mimitin) (Myc-induced mitochondrial protein) (MMTN) (NDUFA12-like protein)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "Chaperone",
"Uniport_ID": "Q8N183",
"Uniprot_name": "NDUF2_HUMAN",
"EC_numbers": "",
"gene_symbol": "NDUFAF2",
"gene_synonyms": "B17.2L, mimitin, MMTN, NDUFA12L",
"gene_synonyms_links": "",
"gene_name": "NADH:ubiquinone oxidoreductase complex assembly factor 2",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000164182",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion",
"reaction": "",
"targ_desciption": "Acts as a molecular chaperone for mitochondrial complex Iassembly (PubMed:16200211, PubMed:19384974). Complex I functions in thetransfer of electrons from NADH to the respiratory chain. The immediateelectron acceptor for the enzyme is believed to be ubiquinone(PubMed:16200211, PubMed:27626371). {ECO:0000269|PubMed:16200211,ECO:0000269|PubMed:19384974, ECO:0000269|PubMed:27626371}.",
"references": []
},
{
"targ_id": "T001032",
"parent_targ_id": "T001",
"full_name": "NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 3",
"abbrev": "",
"protein_names": "NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 3",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "Q9BU61",
"Uniprot_name": "NDUF3_HUMAN",
"EC_numbers": "",
"gene_symbol": "NDUFAF3",
"gene_synonyms": "2P1, C3orf60, DKFZP564J0123, E3-3, MGC10527",
"gene_synonyms_links": "",
"gene_name": "NADH:ubiquinone oxidoreductase complex assembly factor 3",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000178057",
"omim_id": "",
"genecard_link": "",
"locs": "Nucleus; Mitochondrion inner membrane",
"reaction": "",
"targ_desciption": "Essential factor for the assembly of mitochondrialNADH:ubiquinone oxidoreductase complex (complex I).{ECO:0000269|PubMed:19463981}.",
"references": []
},
{
"targ_id": "T001033",
"parent_targ_id": "T001",
"full_name": "NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 4",
"abbrev": "",
"protein_names": "NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 4 (Hormone-regulated proliferation-associated protein of 20 kDa)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "Calmodulin-binding",
"Uniport_ID": "Q9P032",
"Uniprot_name": "NDUF4_HUMAN",
"EC_numbers": "",
"gene_symbol": "NDUFAF4",
"gene_synonyms": "bA22L21.1, C6orf66, HRPAP20, HSPC125, My013",
"gene_synonyms_links": "",
"gene_name": "NADH:ubiquinone oxidoreductase complex assembly factor 4",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000123545",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion; Membrane; Lipid-anchor",
"reaction": "",
"targ_desciption": "Involved in the assembly of mitochondrial NADH:ubiquinoneoxidoreductase complex (complex I) (PubMed:18179882, PubMed:28853723).May be involved in cell proliferation and survival of hormone-dependenttumor cells. May be a regulator of breast tumor cell invasion.{ECO:0000269|PubMed:14871833, ECO:0000269|PubMed:17001319,ECO:0000269|PubMed:18179882, ECO:0000269|PubMed:28853723}.",
"references": []
},
{
"targ_id": "T001034",
"parent_targ_id": "T001",
"full_name": "Arginine-hydroxylase NDUFAF5, mitochondrial",
"abbrev": "",
"protein_names": "Arginine-hydroxylase NDUFAF5, mitochondrial (EC 1.-.-.-) (NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 5) (Putative methyltransferase NDUFAF5) (EC 2.1.1.-)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "Methyltransferase, Oxidoreductase, Transferase",
"Uniport_ID": "Q5TEU4",
"Uniprot_name": "NDUF5_HUMAN",
"EC_numbers": "1.-.-.-; 2.1.1.-",
"gene_symbol": "NDUFAF5",
"gene_synonyms": "C20orf7, dJ842G6.1",
"gene_synonyms_links": "",
"gene_name": "NADH:ubiquinone oxidoreductase complex assembly factor 5",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000101247",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane",
"reaction": "",
"targ_desciption": "Arginine hydroxylase involved in the assembly ofmitochondrial NADH:ubiquinone oxidoreductase complex (complex I, MT-ND1) at early stages (PubMed:18940309, PubMed:27226634). Acts bymediating hydroxylation of 'Arg-111' of NDUFS7 (PubMed:27226634). Mayalso have methyltransferase activity (Probable).{ECO:0000269|PubMed:18940309, ECO:0000269|PubMed:27226634,ECO:0000305}.",
"references": []
},
{
"targ_id": "T001035",
"parent_targ_id": "T001",
"full_name": "NADH dehydrogenase (ubiquinone) complex I, assembly factor 6",
"abbrev": "",
"protein_names": "NADH dehydrogenase (ubiquinone) complex I, assembly factor 6 (Putative phytoene synthase)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "Q330K2",
"Uniprot_name": "NDUF6_HUMAN",
"EC_numbers": "",
"gene_symbol": "NDUFAF6",
"gene_synonyms": "C8orf38, MGC40214",
"gene_synonyms_links": "",
"gene_name": "NADH:ubiquinone oxidoreductase complex assembly factor 6",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000156170",
"omim_id": "",
"genecard_link": "",
"locs": "[Isoform 1]: Mitochondrion inner membrane; [Isoform 2]: Cytoplasm; Nucleus",
"reaction": "",
"targ_desciption": "Involved in the assembly of mitochondrial NADH:ubiquinoneoxidoreductase complex (complex I) at early stages. May play a role inthe biogenesis of MT-ND1. {ECO:0000269|PubMed:18614015,ECO:0000269|PubMed:22019594}.",
"references": []
},
{
"targ_id": "T001036",
"parent_targ_id": "T001",
"full_name": "Protein arginine methyltransferase NDUFAF7, mitochondrial",
"abbrev": "",
"protein_names": "Protein arginine methyltransferase NDUFAF7, mitochondrial (EC 2.1.1.320) (NADH dehydrogenase [ubiquinone] complex I, assembly factor 7) (Protein midA homolog)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "Methyltransferase, Transferase",
"Uniport_ID": "Q7L592",
"Uniprot_name": "NDUF7_HUMAN",
"EC_numbers": "2.1.1.320",
"gene_symbol": "NDUFAF7",
"gene_synonyms": "C2orf56, MidA, PRO1853",
"gene_synonyms_links": "",
"gene_name": "NADH:ubiquinone oxidoreductase complex assembly factor 7",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000003509",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion",
"reaction": "L-arginyl-[protein] + 2 S-adenosyl-L-methionine = 2 H(+) +N(omega),N(omega)'-dimethyl-L-arginyl-[protein] + 2 S-adenosyl-L-homocysteine",
"targ_desciption": "Arginine methyltransferase involved in the assembly orstability of mitochondrial NADH:ubiquinone oxidoreductase complex(complex I) (PubMed:20406883, PubMed:24089531, PubMed:24838397). Actsby mediating symmetric dimethylation of 'Arg-118' of NDUFS2 after itassembles into the complex I, stabilizing the early intermediatecomplex (PubMed:24089531). {ECO:0000269|PubMed:20406883,ECO:0000269|PubMed:24089531, ECO:0000269|PubMed:24838397}.",
"references": []
},
{
"targ_id": "T001037",
"parent_targ_id": "T001",
"full_name": "NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 8",
"abbrev": "",
"protein_names": "NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 8",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "A1L188",
"Uniprot_name": "NDUF8_HUMAN",
"EC_numbers": "",
"gene_symbol": "NDUFAF8",
"gene_synonyms": "C17orf89",
"gene_synonyms_links": "",
"gene_name": "NADH:ubiquinone oxidoreductase complex assembly factor 8",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000224877",
"omim_id": "",
"genecard_link": "",
"locs": "",
"reaction": "",
"targ_desciption": "Involved in the assembly of mitochondrial NADH:ubiquinoneoxidoreductase complex (complex I, MT-ND1) (PubMed:27499296). Requiredto stabilize NDUFAF5 (PubMed:27499296). {ECO:0000269|PubMed:27499296}.",
"references": []
},
{
"targ_id": "T001038",
"parent_targ_id": "T001",
"full_name": "Complex I assembly factor ACAD9, mitochondrial",
"abbrev": "ACAD-9",
"protein_names": "Complex I assembly factor ACAD9, mitochondrial (Acyl-CoA dehydrogenase family member 9) (ACAD-9) (EC 1.3.8.-)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "Oxidoreductase",
"Uniport_ID": "Q9H845",
"Uniprot_name": "ACAD9_HUMAN",
"EC_numbers": "1.3.8.-",
"gene_symbol": "ACAD9",
"gene_synonyms": "MGC14452, NPD002",
"gene_synonyms_links": "",
"gene_name": "Acyl-CoA dehydrogenase family member 9",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000177646",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Peripheral membrane protein; Matrix side",
"reaction": "eicosanoyl-CoA + H(+) + oxidized [electron-transferflavoprotein] = (2E)-eicosenoyl-CoA + reduced [electron-transferflavoprotein]; H(+) + octadecanoyl-CoA + oxidized [electron-transferflavoprotein] = (2E)-octadecenoyl-CoA + reduced [electron-transferflavoprotein]; H(+) + hexadecanoyl-CoA + oxidized [electron-transferflavoprotein] = (2E)-hexadecenoyl-CoA + reduced [electron-transferflavoprotein]; decanoyl-CoA + H(+) + oxidized [electron-transferflavoprotein] = (2E)-decenoyl-CoA + reduced [electron-transferflavoprotein]; H(+) + nonanoyl-CoA + oxidized [electron-transferflavoprotein] = (2E)-nonenoyl-CoA + reduced [electron-transferflavoprotein]; H(+) + oxidized [electron-transfer flavoprotein] +pentadecanoyl-CoA = (2E)-pentadecenoyl-CoA + reduced [electron-transfer flavoprotein]; H(+) + oxidized [electron-transfer flavoprotein] + undecanoyl-CoA = reduced [electron-transfer flavoprotein] + trans-2-undecenoyl-CoA; (9Z)-hexadecenoyl-CoA + H(+) + oxidized [electron-transferflavoprotein] = (2E,9Z)-hexadecadienoyl-CoA + reduced [electron-transfer flavoprotein]; H(+) + heptadecanoyl-CoA + oxidized [electron-transferflavoprotein] = reduced [electron-transfer flavoprotein] + trans-2-heptadecenoyl-CoA; (9E)-octadecenoyl-CoA + H(+) + oxidized [electron-transferflavoprotein] = (2E,9E)-octadecadienoyl-CoA + reduced [electron-transfer flavoprotein]; (9Z)-octadecenoyl-CoA + H(+) + oxidized [electron-transferflavoprotein] = (2E,9Z)-octadecadienoyl-CoA + reduced [electron-transfer flavoprotein]; (9Z,12Z)-octadecadienoyl-CoA + H(+) + oxidized [electron-transfer flavoprotein] = (2E,9Z,12Z)-octadecatrienoyl-CoA + reduced[electron-transfer flavoprotein]; (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoyl-CoA + H(+) + oxidized[electron-transfer flavoprotein] = (2E,4Z,7Z,10Z,13Z,16Z,19Z)-docosaheptaenoyl-CoA + reduced [electron-transfer flavoprotein]; H(+) + oxidized [electron-transfer flavoprotein] +tetradecanoyl-CoA = (2E)-tetradecenoyl-CoA + reduced [electron-transfer flavoprotein]",
"targ_desciption": "As part of the MCIA complex, primarily participates in theassembly of the mitochondrial complex I and therefore plays a role inoxidative phosphorylation (PubMed:20816094, PubMed:24158852). Thismoonlighting protein has also a dehydrogenase activity toward a broadrange of substrates with greater specificity for long-chain unsaturatedacyl-CoAs (PubMed:12359260, PubMed:16020546, PubMed:21237683,PubMed:24158852). However, in vivo, it does not seem to play a primaryrole in fatty acid oxidation (PubMed:20816094, PubMed:24158852). Inaddition, the function in complex I assembly is independent of thedehydrogenase activity of the protein (PubMed:24158852).{ECO:0000269|PubMed:12359260, ECO:0000269|PubMed:16020546,ECO:0000269|PubMed:20816094, ECO:0000269|PubMed:21237683,ECO:0000269|PubMed:24158852}.",
"references": []
},
{
"targ_id": "T001039",
"parent_targ_id": "T001",
"full_name": "Evolutionarily conserved signaling intermediate in Toll pathway, mitochondrial",
"abbrev": "Protein SITPEC",
"protein_names": "Evolutionarily conserved signaling intermediate in Toll pathway, mitochondrial (Protein SITPEC)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "Q9BQ95",
"Uniprot_name": "ECSIT_HUMAN",
"EC_numbers": "",
"gene_symbol": "ECSIT",
"gene_synonyms": "SITPEC",
"gene_synonyms_links": "",
"gene_name": "ECSIT signalling integrator",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000130159",
"omim_id": "",
"genecard_link": "",
"locs": "Cytoplasm; Nucleus; Mitochondrion",
"reaction": "",
"targ_desciption": "Adapter protein of the Toll-like and IL-1 receptor signalingpathway that is involved in the activation of NF-kappa-B via MAP3K1.Promotes proteolytic activation of MAP3K1. Involved in the BMPsignaling pathway. Required for normal embryonic development (Bysimilarity). {ECO:0000250}.; Required for efficient assembly of mitochondrialNADH:ubiquinone oxidoreductase.",
"references": []
},
{
"targ_id": "T001040",
"parent_targ_id": "T001",
"full_name": "FAD-dependent oxidoreductase domain-containing protein 1",
"abbrev": "",
"protein_names": "FAD-dependent oxidoreductase domain-containing protein 1 (EC 1.-.-.-)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "Oxidoreductase",
"Uniport_ID": "Q96CU9",
"Uniprot_name": "FXRD1_HUMAN",
"EC_numbers": "1.-.-.-",
"gene_symbol": "FOXRED1",
"gene_synonyms": "H17",
"gene_synonyms_links": "",
"gene_name": "FAD dependent oxidoreductase domain containing 1",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000110074",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Single-pass membrane protein",
"reaction": "",
"targ_desciption": "Required for the assembly of the mitochondrial membranerespiratory chain NADH dehydrogenase (Complex I) (PubMed:20858599,PubMed:25678554). Involved in mid-late stages of complex I assembly(PubMed:25678554). {ECO:0000269|PubMed:20858599,ECO:0000269|PubMed:25678554}.",
"references": []
},
{
"targ_id": "T001041",
"parent_targ_id": "T001",
"full_name": "Iron-sulfur protein NUBPL",
"abbrev": "huInd1",
"protein_names": "Iron-sulfur protein NUBPL (IND1 homolog) (Nucleotide-binding protein-like) (huInd1)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "Q8TB37",
"Uniprot_name": "NUBPL_HUMAN",
"EC_numbers": "",
"gene_symbol": "NUBPL",
"gene_synonyms": "C14orf127, FLJ12660, huInd1, IND1",
"gene_synonyms_links": "",
"gene_name": "Nucleotide binding protein like",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000151413",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion",
"reaction": "",
"targ_desciption": "Required for the assembly of the mitochondrial membranerespiratory chain NADH dehydrogenase (Complex I). May deliver of one ormore Fe-S clusters to complex I subunits.{ECO:0000269|PubMed:19752196}.",
"references": []
},
{
"targ_id": "T001042",
"parent_targ_id": "T001",
"full_name": "Complex I assembly factor TIMMDC1, mitochondrial",
"abbrev": "Protein M5-14",
"protein_names": "Complex I assembly factor TIMMDC1, mitochondrial (Protein M5-14) (Translocase of inner mitochondrial membrane domain-containing protein 1) (TIMM domain containing-protein 1)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "Chaperone",
"Uniport_ID": "Q9NPL8",
"Uniprot_name": "TIDC1_HUMAN",
"EC_numbers": "",
"gene_symbol": "TIMMDC1",
"gene_synonyms": "C3orf1, FLJ22597",
"gene_synonyms_links": "",
"gene_name": "Translocase of inner mitochondrial membrane domain containing 1",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000113845",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion membrane; Multi-pass membrane protein",
"reaction": "",
"targ_desciption": "Chaperone protein involved in the assembly of themitochondrial NADH:ubiquinone oxidoreductase complex (complex I).Participates in constructing the membrane arm of complex I.{ECO:0000269|PubMed:24191001}.",
"references": []
},
{
"targ_id": "T001043",
"parent_targ_id": "T001",
"full_name": "Complex I assembly factor TMEM126B, mitochondrial",
"abbrev": "",
"protein_names": "Complex I assembly factor TMEM126B, mitochondrial (Transmembrane protein 126B)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "Chaperone",
"Uniport_ID": "Q8IUX1",
"Uniprot_name": "T126B_HUMAN",
"EC_numbers": "",
"gene_symbol": "TMEM126B",
"gene_synonyms": "HT007",
"gene_synonyms_links": "",
"gene_name": "Transmembrane protein 126B",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000171204",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion membrane; Multi-pass membrane protein",
"reaction": "",
"targ_desciption": "Chaperone protein involved in the assembly of themitochondrial NADH:ubiquinone oxidoreductase complex (complex I).Participates in constructing the membrane arm of complex I.{ECO:0000269|PubMed:24191001, ECO:0000269|PubMed:27374773,ECO:0000269|PubMed:27374774}.",
"references": []
},
{
"targ_id": "T001044",
"parent_targ_id": "T001",
"full_name": "NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6",
"abbrev": "CI-B17",
"protein_names": "NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 (Complex I-B17) (CI-B17) (NADH-ubiquinone oxidoreductase B17 subunit)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "O95139",
"Uniprot_name": "NDUB6_HUMAN",
"EC_numbers": "",
"gene_symbol": "NDUFB6",
"gene_synonyms": "B17, CI",
"gene_synonyms_links": "",
"gene_name": "NADH:ubiquinone oxidoreductase subunit B6",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000165264",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Single-pass membrane protein; Matrix side",
"reaction": "",
"targ_desciption": "Accessory subunit of the mitochondrial membrane respiratorychain NADH dehydrogenase (Complex I), that is believed not to beinvolved in catalysis. Complex I functions in the transfer of electronsfrom NADH to the respiratory chain. The immediate electron acceptor forthe enzyme is believed to be ubiquinone. {ECO:0000269|PubMed:27626371}.",
"references": []
},
{
"targ_id": "T001045",
"parent_targ_id": "T001",
"full_name": "NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5",
"abbrev": "CI-13kD-B",
"protein_names": "NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 (Complex I subunit B13) (Complex I-13kD-B) (CI-13kD-B) (NADH-ubiquinone oxidoreductase 13 kDa-B subunit)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "Q16718",
"Uniprot_name": "NDUA5_HUMAN",
"EC_numbers": "",
"gene_symbol": "NDUFA5",
"gene_synonyms": "B13, CI-13kB, CI-13KD-B, NUFM, UQOR13",
"gene_synonyms_links": "",
"gene_name": "NADH:ubiquinone oxidoreductase subunit A5",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000128609",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Peripheral membrane protein; Matrix side",
"reaction": "",
"targ_desciption": "Accessory subunit of the mitochondrial membrane respiratorychain NADH dehydrogenase (Complex I), that is believed not to beinvolved in catalysis. Complex I functions in the transfer of electronsfrom NADH to the respiratory chain. The immediate electron acceptor forthe enzyme is believed to be ubiquinone. {ECO:0000269|PubMed:27626371}.",
"references": []
},
{
"targ_id": "T001046",
"parent_targ_id": "T001",
"full_name": "NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial",
"abbrev": "CI-13kD-A",
"protein_names": "NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial (Complex I-13kD-A) (CI-13kD-A) (NADH-ubiquinone oxidoreductase 13 kDa-A subunit)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "O75380",
"Uniprot_name": "NDUS6_HUMAN",
"EC_numbers": "",
"gene_symbol": "NDUFS6",
"gene_synonyms": "CI-13kA",
"gene_synonyms_links": "",
"gene_name": "NADH:ubiquinone oxidoreductase subunit S6",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000145494",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Peripheral membrane protein; Matrix side",
"reaction": "",
"targ_desciption": "Accessory subunit of the mitochondrial membrane respiratorychain NADH dehydrogenase (Complex I), that is believed not to beinvolved in catalysis. Complex I functions in the transfer of electronsfrom NADH to the respiratory chain. The immediate electron acceptor forthe enzyme is believed to be ubiquinone. {ECO:0000269|PubMed:27626371}.",
"references": []
},
{
"targ_id": "T001047",
"parent_targ_id": "T001",
"full_name": "NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1",
"abbrev": "CI-MNLL",
"protein_names": "NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 (Complex I-MNLL) (CI-MNLL) (NADH-ubiquinone oxidoreductase MNLL subunit)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "O75438",
"Uniprot_name": "NDUB1_HUMAN",
"EC_numbers": "",
"gene_symbol": "NDUFB1",
"gene_synonyms": "CI-MNLL, MNLL",
"gene_synonyms_links": "",
"gene_name": "NADH:ubiquinone oxidoreductase subunit B1",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000183648",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Single-pass membrane protein; Matrix side",
"reaction": "",
"targ_desciption": "Accessory subunit of the mitochondrial membrane respiratorychain NADH dehydrogenase (Complex I) that is believed not to beinvolved in catalysis. Complex I functions in the transfer of electronsfrom NADH to the respiratory chain. The immediate electron acceptor forthe enzyme is believed to be ubiquinone. {ECO:0000269|PubMed:27626371}.",
"references": []
},
{
"targ_id": "T001048",
"parent_targ_id": "T001",
"full_name": "NADH dehydrogenase [ubiquinone] iron-sulfur protein 5",
"abbrev": "CI-15 kDa",
"protein_names": "NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 (Complex I-15 kDa) (CI-15 kDa) (NADH-ubiquinone oxidoreductase 15 kDa subunit)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "O43920",
"Uniprot_name": "NDUS5_HUMAN",
"EC_numbers": "",
"gene_symbol": "NDUFS5",
"gene_synonyms": "CI-15k",
"gene_synonyms_links": "",
"gene_name": "NADH:ubiquinone oxidoreductase subunit S5",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000168653",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Peripheral membrane protein; Mitochondrion intermembrane space",
"reaction": "",
"targ_desciption": "Accessory subunit of the mitochondrial membrane respiratorychain NADH dehydrogenase (Complex I), that is believed not to beinvolved in catalysis. Complex I functions in the transfer of electronsfrom NADH to the respiratory chain. The immediate electron acceptor forthe enzyme is believed to be ubiquinone. {ECO:0000269|PubMed:27626371}.",
"references": []
},
{
"targ_id": "T001049",
"parent_targ_id": "T001",
"full_name": "NADH dehydrogenase [ubiquinone] 1 subunit C2",
"abbrev": "CI-B14.5b",
"protein_names": "NADH dehydrogenase [ubiquinone] 1 subunit C2 (Complex I-B14.5b) (CI-B14.5b) (Human lung cancer oncogene 1 protein) (HLC-1) (NADH-ubiquinone oxidoreductase subunit B14.5b)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "O95298",
"Uniprot_name": "NDUC2_HUMAN",
"EC_numbers": "",
"gene_symbol": "NDUFC2",
"gene_synonyms": "B14.5b, HLC-1",
"gene_synonyms_links": "",
"gene_name": "NADH:ubiquinone oxidoreductase subunit C2",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000151366",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Single-pass membrane protein; Matrix side",
"reaction": "",
"targ_desciption": "Accessory subunit of the mitochondrial membrane respiratorychain NADH dehydrogenase (Complex I), that is believed not to beinvolved in catalysis. Complex I functions in the transfer of electronsfrom NADH to the respiratory chain. The immediate electron acceptor forthe enzyme is believed to be ubiquinone. {ECO:0000269|PubMed:27626371}.",
"references": []
},
{
"targ_id": "T001050",
"parent_targ_id": "T001",
"full_name": "NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7",
"abbrev": "CI-B14.5a",
"protein_names": "NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 (Complex I-B14.5a) (CI-B14.5a) (NADH-ubiquinone oxidoreductase subunit B14.5a)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "O95182",
"Uniprot_name": "NDUA7_HUMAN",
"EC_numbers": "",
"gene_symbol": "NDUFA7",
"gene_synonyms": "B14.5a",
"gene_synonyms_links": "",
"gene_name": "NADH:ubiquinone oxidoreductase subunit A7",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000267855",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Peripheral membrane protein; Matrix side",
"reaction": "",
"targ_desciption": "Accessory subunit of the mitochondrial membrane respiratorychain NADH dehydrogenase (Complex I), that is believed not to beinvolved in catalysis. Complex I functions in the transfer of electronsfrom NADH to the respiratory chain. The immediate electron acceptor forthe enzyme is believed to be ubiquinone. {ECO:0000269|PubMed:27626371}.",
"references": []
},
{
"targ_id": "T001051",
"parent_targ_id": "T001",
"full_name": "NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8",
"abbrev": "CI-19kD",
"protein_names": "NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 (Complex I-19kD) (CI-19kD) (Complex I-PGIV) (CI-PGIV) (NADH-ubiquinone oxidoreductase 19 kDa subunit)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "P51970",
"Uniprot_name": "NDUA8_HUMAN",
"EC_numbers": "",
"gene_symbol": "NDUFA8",
"gene_synonyms": "MGC793, PGIV",
"gene_synonyms_links": "",
"gene_name": "NADH:ubiquinone oxidoreductase subunit A8",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000119421",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Peripheral membrane protein; Mitochondrion intermembrane space; Mitochondrion",
"reaction": "",
"targ_desciption": "Accessory subunit of the mitochondrial membrane respiratorychain NADH dehydrogenase (Complex I), that is believed not to beinvolved in catalysis. Complex I functions in the transfer of electronsfrom NADH to the respiratory chain. The immediate electron acceptor forthe enzyme is believed to be ubiquinone. {ECO:0000269|PubMed:27626371}.",
"references": []
},
{
"targ_id": "T001052",
"parent_targ_id": "T001",
"full_name": "NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7",
"abbrev": "CI-B18",
"protein_names": "NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 (Cell adhesion protein SQM1) (Complex I-B18) (CI-B18) (NADH-ubiquinone oxidoreductase B18 subunit)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "P17568",
"Uniprot_name": "NDUB7_HUMAN",
"EC_numbers": "",
"gene_symbol": "NDUFB7",
"gene_synonyms": "B18, CI-B18, MGC2480",
"gene_synonyms_links": "",
"gene_name": "NADH:ubiquinone oxidoreductase subunit B7",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000099795",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Peripheral membrane protein; Mitochondrion intermembrane space",
"reaction": "",
"targ_desciption": "Accessory subunit of the mitochondrial membrane respiratorychain NADH dehydrogenase (Complex I), that is believed not to beinvolved in catalysis. Complex I functions in the transfer of electronsfrom NADH to the respiratory chain. The immediate electron acceptor forthe enzyme is believed to be ubiquinone. {ECO:0000269|PubMed:27626371}.",
"references": []
},
{
"targ_id": "T001053",
"parent_targ_id": "T001",
"full_name": "NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial",
"abbrev": "CI-AGGG",
"protein_names": "NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial (Complex I-AGGG) (CI-AGGG) (NADH-ubiquinone oxidoreductase AGGG subunit)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "O95178",
"Uniprot_name": "NDUB2_HUMAN",
"EC_numbers": "",
"gene_symbol": "NDUFB2",
"gene_synonyms": "AGGG, CI-AGGG",
"gene_synonyms_links": "",
"gene_name": "NADH:ubiquinone oxidoreductase subunit B2",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000090266",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Peripheral membrane protein; Matrix side",
"reaction": "",
"targ_desciption": "Accessory subunit of the mitochondrial membrane respiratorychain NADH dehydrogenase (Complex I), that is believed not to beinvolved in catalysis. Complex I functions in the transfer of electronsfrom NADH to the respiratory chain. The immediate electron acceptor forthe enzyme is believed to be ubiquinone. {ECO:0000269|PubMed:27626371}.",
"references": []
},
{
"targ_id": "T001054",
"parent_targ_id": "T001",
"full_name": "NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3",
"abbrev": "CI-B9",
"protein_names": "NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 (Complex I-B9) (CI-B9) (NADH-ubiquinone oxidoreductase B9 subunit)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "O95167",
"Uniprot_name": "NDUA3_HUMAN",
"EC_numbers": "",
"gene_symbol": "NDUFA3",
"gene_synonyms": "B9",
"gene_synonyms_links": "",
"gene_name": "NADH:ubiquinone oxidoreductase subunit A3",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000170906",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Single-pass membrane protein",
"reaction": "",
"targ_desciption": "Accessory subunit of the mitochondrial membrane respiratorychain NADH dehydrogenase (Complex I), that is believed not to beinvolved in catalysis. Complex I functions in the transfer of electronsfrom NADH to the respiratory chain. The immediate electron acceptor forthe enzyme is believed to be ubiquinone. {ECO:0000269|PubMed:27626371}.",
"references": []
},
{
"targ_id": "T001055",
"parent_targ_id": "T001",
"full_name": "NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial",
"abbrev": "CI-SGDH",
"protein_names": "NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial (Complex I-SGDH) (CI-SGDH) (NADH-ubiquinone oxidoreductase SGDH subunit)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "O43674",
"Uniprot_name": "NDUB5_HUMAN",
"EC_numbers": "",
"gene_symbol": "NDUFB5",
"gene_synonyms": "CI-SGDH, MGC12314, SGDH",
"gene_synonyms_links": "",
"gene_name": "NADH:ubiquinone oxidoreductase subunit B5",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000136521",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Single-pass membrane protein; Matrix side",
"reaction": "",
"targ_desciption": "Accessory subunit of the mitochondrial membrane respiratorychain NADH dehydrogenase (Complex I), that is believed not to beinvolved in catalysis. Complex I functions in the transfer of electronsfrom NADH to the respiratory chain. The immediate electron acceptor forthe enzyme is believed to be ubiquinone. {ECO:0000269|PubMed:27626371}.",
"references": []
},
{
"targ_id": "T001056",
"parent_targ_id": "T001",
"full_name": "NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4",
"abbrev": "CI-B15",
"protein_names": "NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 (Complex I-B15) (CI-B15) (NADH-ubiquinone oxidoreductase B15 subunit)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "O95168",
"Uniprot_name": "NDUB4_HUMAN",
"EC_numbers": "",
"gene_symbol": "NDUFB4",
"gene_synonyms": "B15",
"gene_synonyms_links": "",
"gene_name": "NADH:ubiquinone oxidoreductase subunit B4",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000065518",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Single-pass membrane protein; Matrix side",
"reaction": "",
"targ_desciption": "Accessory subunit of the mitochondrial membrane respiratorychain NADH dehydrogenase (Complex I), that is believed not to beinvolved in catalysis. Complex I functions in the transfer of electronsfrom NADH to the respiratory chain. The immediate electron acceptor forthe enzyme is believed to be ubiquinone. {ECO:0000269|PubMed:27626371}.",
"references": []
},
{
"targ_id": "T001057",
"parent_targ_id": "T001",
"full_name": "NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial",
"abbrev": "CI-KFYI",
"protein_names": "NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial (Complex I-KFYI) (CI-KFYI) (NADH-ubiquinone oxidoreductase KFYI subunit)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "O43677",
"Uniprot_name": "NDUC1_HUMAN",
"EC_numbers": "",
"gene_symbol": "NDUFC1",
"gene_synonyms": "KFYI",
"gene_synonyms_links": "",
"gene_name": "NADH:ubiquinone oxidoreductase subunit C1",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000109390",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Single-pass membrane protein; Matrix side",
"reaction": "",
"targ_desciption": "Accessory subunit of the mitochondrial membrane respiratorychain NADH dehydrogenase (Complex I), that is believed not to beinvolved in catalysis. Complex I functions in the transfer of electronsfrom NADH to the respiratory chain. The immediate electron acceptor forthe enzyme is believed to be ubiquinone. {ECO:0000269|PubMed:27626371}.",
"references": []
},
{
"targ_id": "T001058",
"parent_targ_id": "T001",
"full_name": "NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial",
"abbrev": "CI-9kD",
"protein_names": "NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial (Complex I-9kD) (CI-9kD) (NADH-ubiquinone oxidoreductase 9 kDa subunit) (Renal carcinoma antigen NY-REN-4)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "P56181",
"Uniprot_name": "NDUV3_HUMAN",
"EC_numbers": "",
"gene_symbol": "NDUFV3",
"gene_synonyms": "CI-10k",
"gene_synonyms_links": "",
"gene_name": "NADH:ubiquinone oxidoreductase subunit V3",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000160194",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Peripheral membrane protein; Matrix side",
"reaction": "",
"targ_desciption": "Accessory subunit of the mitochondrial membrane respiratorychain NADH dehydrogenase (Complex I), that is believed not to beinvolved in catalysis. Complex I functions in the transfer of electronsfrom NADH to the respiratory chain. The immediate electron acceptor forthe enzyme is believed to be ubiquinone. May be the terminallyassembled subunit of Complex I. {ECO:0000269|PubMed:27626371}.",
"references": []
},
{
"targ_id": "T001059",
"parent_targ_id": "T001",
"full_name": "Acyl carrier protein, mitochondrial",
"abbrev": "CI-SDAP",
"protein_names": "Acyl carrier protein, mitochondrial (ACP) (CI-SDAP) (NADH-ubiquinone oxidoreductase 9.6 kDa subunit)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "O14561",
"Uniprot_name": "ACPM_HUMAN",
"EC_numbers": "",
"gene_symbol": "NDUFAB1",
"gene_synonyms": "ACP, ACP1, FASN2A, SDAP",
"gene_synonyms_links": "",
"gene_name": "NADH:ubiquinone oxidoreductase subunit AB1",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000004779",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion",
"reaction": "",
"targ_desciption": "Carrier of the growing fatty acid chain in fatty acidbiosynthesis (By similarity). Accessory and non-catalytic subunit ofthe mitochondrial membrane respiratory chain NADH dehydrogenase(Complex I), which functions in the transfer of electrons from NADH tothe respiratory chain (PubMed:27626371). {ECO:0000250|UniProtKB:P52505,ECO:0000269|PubMed:27626371}.",
"references": []
},
{
"targ_id": "T001060",
"parent_targ_id": "T001",
"full_name": "quinone",
"abbrev": "quinone",
"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": [
"RC01100",
"RC01101",
"RC01102"
]
},
{
"targ_id": "T001061",
"parent_targ_id": "T001",
"full_name": "semiquinone",
"abbrev": "semiquinone",
"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": [
"RC01103",
"RC01104",
"RC01105",
"RC01106",
"RC01107",
"RC01108",
"RC01109",
"RC01110",
"RC03655"
]
},
{
"targ_id": "T001062",
"parent_targ_id": "T001",
"full_name": "quinol",
"abbrev": "quinol",
"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": [
"RC01111",
"RC01112",
"RC01113",
"RC01114",
"RC01115",
"RC01116",
"RC01117",
"RC01118"
]
},
{
"targ_id": "T001063",
"parent_targ_id": "T001",
"full_name": "flavin mononucleotide",
"abbrev": "FMN",
"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": [
"RC01119"
]
},
{
"targ_id": "T001064",
"parent_targ_id": "T001",
"full_name": "iron-sulfur clusters",
"abbrev": "iron-sulfur clusters",
"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": [
"RC01120",
"RC01147"
]
},
{
"targ_id": "T002",
"parent_targ_id": "T",
"full_name": "Succinate dehydrogenase",
"abbrev": "Complex II",
"protein_names": "",
"related_func_ids": "F020103; F0203",
"category": "enzyme",
"subcategories": "",
"Uniport_ID": "",
"Uniprot_name": "",
"EC_numbers": "1.3.5.1",
"gene_symbol": "",
"gene_synonyms": "",
"gene_synonyms_links": "",
"gene_name": "",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "",
"omim_id": "",
"genecard_link": "",
"locs": "",
"reaction": "",
"targ_desciption": "",
"references": [
"RC00113",
"RC00118",
"RC00123",
"RC00128",
"RC00133",
"RC00138",
"RC00143",
"RC00148",
"RC00153",
"RC00158",
"RC00163",
"RC00168",
"RC00173",
"RC00178",
"RC00183",
"RC00188",
"RC00193",
"RC00198",
"RC00203",
"RC00208",
"RC00213",
"RC00218",
"RC00919",
"RC00922",
"RC00942",
"RC00954",
"RC00971",
"RC01054",
"RC01121",
"RC03918",
"RC03954",
"RC03955",
"RC03987",
"RC03988",
"RC03989",
"RC03990",
"RC04009",
"RC04516",
"RC04517",
"RC04518",
"RC04519",
"RC04520",
"RC04521",
"RC04524",
"RC04525",
"RC04526",
"RC04527",
"RC04528",
"RC04529",
"RC04530",
"RC04531",
"RC04532",
"RC04533",
"RC04534",
"RC04536",
"RC04537",
"RC04538",
"RC04539",
"RC04540",
"RC04542",
"RC04545",
"RC04546",
"RC04547",
"RC04548",
"RC04549",
"RC04552",
"RC04559",
"RC04560",
"RC04561",
"RC04563",
"RC04564",
"RC04566",
"RC04567",
"RC04569",
"RC04570",
"RC04572",
"RC04573",
"RC04574",
"RC04575",
"RC04577",
"RC04578",
"RC04579",
"RC04580",
"RC04581",
"RC04583",
"RC04587",
"RC04588",
"RC04593",
"RC04594",
"RC04596",
"RC04597",
"RC04601",
"RC04604",
"RC04605",
"RC04606",
"RC04608",
"RC04610",
"RC04611",
"RC04612",
"RC04613",
"RC04616",
"RC04618",
"RC04619",
"RC04624",
"RC04627",
"RC04631",
"RC04634",
"RC04636",
"RC04734",
"RC04910",
"RC04918",
"RC04959"
]
},
{
"targ_id": "T002001",
"parent_targ_id": "T002",
"full_name": "Succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial",
"abbrev": "CybS",
"protein_names": "Succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial (CybS) (CII-4) (QPs3) (Succinate dehydrogenase complex subunit D) (Succinate-ubiquinone oxidoreductase cytochrome b small subunit) (Succinate-ubiquinone reductase membrane anchor subunit)",
"related_func_ids": "F020103; F0203",
"category": "protein subunit",
"subcategories": "Oxidoreductase",
"Uniport_ID": "O14521",
"Uniprot_name": "DHSD_HUMAN",
"EC_numbers": "1.3.5.1",
"gene_symbol": "SDHD",
"gene_synonyms": "cybS, PGL, PGL1",
"gene_synonyms_links": "",
"gene_name": "Succinate dehydrogenase complex subunit D",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000204370",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Multi-pass membrane protein",
"reaction": "",
"targ_desciption": "Membrane-anchoring subunit of succinate dehydrogenase (SDH)that is involved in complex II of the mitochondrial electron transportchain and is responsible for transferring electrons from succinate toubiquinone (coenzyme Q). {ECO:0000250}.",
"references": []
},
{
"targ_id": "T002002",
"parent_targ_id": "T002",
"full_name": "Succinate dehydrogenase cytochrome b560 subunit, mitochondrial",
"abbrev": "QPs-1",
"protein_names": "Succinate dehydrogenase cytochrome b560 subunit, mitochondrial (Integral membrane protein CII-3) (QPs-1) (QPs1) (Succinate dehydrogenase complex subunit C) (Succinate-ubiquinone oxidoreductase cytochrome B large subunit) (CYBL)",
"related_func_ids": "F020103; F0203",
"category": "protein subunit",
"subcategories": "Oxidoreductase",
"Uniport_ID": "Q99643",
"Uniprot_name": "C560_HUMAN",
"EC_numbers": "1.3.5.1",
"gene_symbol": "SDHC",
"gene_synonyms": "CYB560, cybL, PGL3",
"gene_synonyms_links": "",
"gene_name": "Succinate dehydrogenase complex subunit C",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000143252",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Multi-pass membrane protein",
"reaction": "",
"targ_desciption": "Membrane-anchoring subunit of succinate dehydrogenase (SDH)that is involved in complex II of the mitochondrial electron transportchain and is responsible for transferring electrons from succinate toubiquinone (coenzyme Q).",
"references": []
},
{
"targ_id": "T002003",
"parent_targ_id": "T002",
"full_name": "Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial",
"abbrev": "Fp",
"protein_names": "Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial (EC 1.3.5.1) (Flavoprotein subunit of complex II) (Fp)",
"related_func_ids": "F020103; F0203",
"category": "protein subunit",
"subcategories": "Chaperone",
"Uniport_ID": "P31040",
"Uniprot_name": "SDHA_HUMAN",
"EC_numbers": "1.3.5.1",
"gene_symbol": "SDHA",
"gene_synonyms": "FP, SDH2, SDHF",
"gene_synonyms_links": "",
"gene_name": "Succinate dehydrogenase complex flavoprotein subunit A",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000073578",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Peripheral membrane protein; Matrix side",
"reaction": "a quinone + succinate = a quinol + fumarate",
"targ_desciption": "Flavoprotein (FP) subunit of succinate dehydrogenase (SDH)that is involved in complex II of the mitochondrial electron transportchain and is responsible for transferring electrons from succinate toubiquinone (coenzyme Q) (PubMed:24781757). Can act as a tumorsuppressor (PubMed:20484225). {ECO:0000269|PubMed:20484225,ECO:0000305|PubMed:24781757}.",
"references": []
},
{
"targ_id": "T002004",
"parent_targ_id": "T002",
"full_name": "Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial",
"abbrev": "Ip",
"protein_names": "Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial (EC 1.3.5.1) (Iron-sulfur subunit of complex II) (Ip)",
"related_func_ids": "F020103; F0203",
"category": "protein subunit",
"subcategories": "Chaperone",
"Uniport_ID": "P21912",
"Uniprot_name": "SDHB_HUMAN",
"EC_numbers": "1.3.5.1",
"gene_symbol": "SDHB",
"gene_synonyms": "SDH, SDH1",
"gene_synonyms_links": "",
"gene_name": "Succinate dehydrogenase complex iron sulfur subunit B",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000117118",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Peripheral membrane protein; Matrix side",
"reaction": "a quinone + succinate = a quinol + fumarate",
"targ_desciption": "Iron-sulfur protein (IP) subunit of succinate dehydrogenase(SDH) that is involved in complex II of the mitochondrial electrontransport chain and is responsible for transferring electrons fromsuccinate to ubiquinone (coenzyme Q).",
"references": []
},
{
"targ_id": "T002005",
"parent_targ_id": "T002",
"full_name": "Succinate dehydrogenase assembly factor 1, mitochondrial",
"abbrev": "SDHAF1",
"protein_names": "Succinate dehydrogenase assembly factor 1, mitochondrial (SDH assembly factor 1) (SDHAF1) (LYR motif-containing protein 8)",
"related_func_ids": "F020103; F0203",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "A6NFY7",
"Uniprot_name": "SDHF1_HUMAN",
"EC_numbers": "1.3.5.1",
"gene_symbol": "SDHAF1",
"gene_synonyms": "LYRM8",
"gene_synonyms_links": "",
"gene_name": "Succinate dehydrogenase complex assembly factor 1",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000205138",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion matrix",
"reaction": "",
"targ_desciption": "Plays an essential role in the assembly of succinatedehydrogenase (SDH), an enzyme complex (also referred to as respiratorycomplex II) that is a component of both the tricarboxylic acid (TCA)cycle and the mitochondrial electron transport chain, and which couplesthe oxidation of succinate to fumarate with the reduction of ubiquinone(coenzyme Q) to ubiquinol (PubMed:24954417, PubMed:19465911). Promotesmaturation of the iron-sulfur protein subunit SDHB of the SDH catalyticdimer, protecting it from the deleterious effects of oxidants(PubMed:24954417). May act together with SDHAF3 (PubMed:24954417).Contributes to iron-sulfur cluster incorporation into SDHB by bindingto SDHB and recruiting the iron-sulfur transfer complex formed byHSC20, HSPA9 and ISCU through direct binding to HSC20(PubMed:26749241). {ECO:0000269|PubMed:19465911,ECO:0000269|PubMed:24954417, ECO:0000269|PubMed:26749241}.",
"references": []
},
{
"targ_id": "T002006",
"parent_targ_id": "T002",
"full_name": "Succinate dehydrogenase assembly factor 2, mitochondrial",
"abbrev": "SDHAF2",
"protein_names": "Succinate dehydrogenase assembly factor 2, mitochondrial (SDH assembly factor 2) (SDHAF2)",
"related_func_ids": "F020103; F0203",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "Q9NX18",
"Uniprot_name": "SDHF2_HUMAN",
"EC_numbers": "1.3.5.1",
"gene_symbol": "SDHAF2",
"gene_synonyms": "C11orf79, FLJ20487, PGL2, SDH5",
"gene_synonyms_links": "",
"gene_name": "Succinate dehydrogenase complex assembly factor 2",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000167985",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion matrix",
"reaction": "",
"targ_desciption": "Plays an essential role in the assembly of succinatedehydrogenase (SDH), an enzyme complex (also referred to as respiratorycomplex II) that is a component of both the tricarboxylic acid (TCA)cycle and the mitochondrial electron transport chain, and which couplesthe oxidation of succinate to fumarate with the reduction of ubiquinone(coenzyme Q) to ubiquinol. Required for flavinylation (covalentattachment of FAD) of the flavoprotein subunit SDHA of the SDHcatalytic dimer. {ECO:0000255|HAMAP-Rule:MF_03057,ECO:0000269|PubMed:19628817}.",
"references": []
},
{
"targ_id": "T002007",
"parent_targ_id": "T002",
"full_name": "Quinone pocket",
"abbrev": "Quinone pocket",
"protein_names": "",
"related_func_ids": "F020103; F0203",
"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": [
"RC01123",
"RC01124",
"RC01125",
"RC01126",
"RC01127",
"RC01128",
"RC01129",
"RC01130",
"RC01131",
"RC01132",
"RC01138",
"RC01139",
"RC01140",
"RC03573",
"RC03574",
"RC03575",
"RC03576"
]
},
{
"targ_id": "T002008",
"parent_targ_id": "T002",
"full_name": "Succinate pocket",
"abbrev": "Succinate pocket",
"protein_names": "",
"related_func_ids": "F020103; F0203",
"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": [
"RC01141",
"RC01142"
]
},
{
"targ_id": "T002009",
"parent_targ_id": "T002",
"full_name": "Succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial",
"abbrev": "CybS",
"protein_names": "Succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial, CybS (CII-4) (QPs3) (Succinate dehydrogenase complex subunit D) (Succinate-ubiquinone oxidoreductase cytochrome b small subunit) (Succinate-ubiquinone reductase membrane anchor subunit) ",
"related_func_ids": "F0203",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "O14521",
"Uniprot_name": "DHSD_HUMAN",
"EC_numbers": "",
"gene_symbol": "SDHD",
"gene_synonyms": "cybS, PGL, PGL1",
"gene_synonyms_links": "",
"gene_name": "Succinate dehydrogenase complex subunit D",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000204370",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane ; Multi-pass membrane protein",
"reaction": "",
"targ_desciption": "Component of the ubiquinol-cytochrome c reductase complex (complex III or cytochrome b-c1 complex) that is part of the mitochondrial respiratory chain. The b-c1 complex mediates electron transfer from ubiquinol to cytochrome c. Contributes to the generation of a proton gradient across the mitochondrial membrane that is then used for ATP synthesis.",
"references": [
"RC01143",
"RC01144",
"RC01145",
"RC01146"
]
},
{
"targ_id": "T003",
"parent_targ_id": "T",
"full_name": "coenzyme Q10",
"abbrev": "",
"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": [
"RC04010"
]
},
{
"targ_id": "T004",
"parent_targ_id": "T",
"full_name": "Quinol--cytochrome-c reductase",
"abbrev": "Complex III",
"protein_names": "",
"related_func_ids": "F020103",
"category": "enzyme",
"subcategories": "",
"Uniport_ID": "",
"Uniprot_name": "",
"EC_numbers": "7.1.1.8",
"gene_symbol": "",
"gene_synonyms": "",
"gene_synonyms_links": "",
"gene_name": "",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "",
"omim_id": "",
"genecard_link": "",
"locs": "",
"reaction": "2 [Fe(III)cytochrome c](out) + a quinol = 2 [Fe(II)cytochromec](out) + a quinone + 2 H(+)(out)",
"targ_desciption": "",
"references": [
"RC00114",
"RC00119",
"RC00124",
"RC00129",
"RC00134",
"RC00139",
"RC00144",
"RC00149",
"RC00154",
"RC00159",
"RC00164",
"RC00169",
"RC00174",
"RC00179",
"RC00184",
"RC00189",
"RC00194",
"RC00199",
"RC00204",
"RC00209",
"RC00214",
"RC00219",
"RC00909",
"RC00943",
"RC00948",
"RC00950",
"RC00955",
"RC00963",
"RC00967",
"RC01122",
"RC01174",
"RC01175",
"RC01176",
"RC01177",
"RC03461",
"RC03462",
"RC03463",
"RC03464",
"RC03651",
"RC03652",
"RC03659",
"RC03856",
"RC03857",
"RC03913",
"RC03976",
"RC03977",
"RC04761",
"RC04762",
"RC04907",
"RC04909",
"RC04911",
"RC04950",
"RC04951"
]
},
{
"targ_id": "T004001",
"parent_targ_id": "T004",
"full_name": "Cytochrome b",
"abbrev": "Complex III subunit 3",
"protein_names": "Cytochrome b (Complex III subunit 3) (Complex III subunit III) (Cytochrome b-c1 complex subunit 3) (Ubiquinol-cytochrome-c reductase complex cytochrome b subunit)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "P00156",
"Uniprot_name": "CYB_HUMAN",
"EC_numbers": "",
"gene_symbol": "MT-CYB",
"gene_synonyms": "COB, CYTB, MTCYB, UQCR3",
"gene_synonyms_links": "",
"gene_name": "Mitochondrially encoded cytochrome b",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000198727",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Multi-pass membrane protein",
"reaction": "",
"targ_desciption": "Component of the ubiquinol-cytochrome c reductase complex(complex III or cytochrome b-c1 complex) that is part of themitochondrial respiratory chain. The b-c1 complex mediates electrontransfer from ubiquinol to cytochrome c. Contributes to the generationof a proton gradient across the mitochondrial membrane that is thenused for ATP synthesis. {ECO:0000250|UniProtKB:P00157}.",
"references": [
"RC00915",
"RC03439",
"RC03440"
]
},
{
"targ_id": "T004002",
"parent_targ_id": "T004",
"full_name": "Cytochrome b-c1 complex subunit 2, mitochondrial",
"abbrev": "Complex III subunit 2",
"protein_names": "Cytochrome b-c1 complex subunit 2, mitochondrial (Complex III subunit 2) (Core protein II) (Ubiquinol-cytochrome-c reductase complex core protein 2)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "P22695",
"Uniprot_name": "QCR2_HUMAN",
"EC_numbers": "",
"gene_symbol": "UQCRC2",
"gene_synonyms": "QCR2, UQCR2",
"gene_synonyms_links": "",
"gene_name": "Ubiquinol-cytochrome c reductase core protein 2",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000140740",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Peripheral membrane protein; Matrix side",
"reaction": "",
"targ_desciption": "Component of the ubiquinol-cytochrome c oxidoreductase, amultisubunit transmembrane complex that is part of the mitochondrialelectron transport chain which drives oxidative phosphorylation. Therespiratory chain contains 3 multisubunit complexes succinatedehydrogenase (complex II, CII), ubiquinol-cytochrome c oxidoreductase(cytochrome b-c1 complex, complex III, CIII) and cytochrome c oxidase(complex IV, CIV), that cooperate to transfer electrons derived fromNADH and succinate to molecular oxygen, creating an electrochemicalgradient over the inner membrane that drives transmembrane transportand the ATP synthase. The cytochrome b-c1 complex catalyzes electrontransfer from ubiquinol to cytochrome c, linking this redox reaction totranslocation of protons across the mitochondrial inner membrane, withprotons being carried across the membrane as hydrogens on the quinol.In the process called Q cycle, 2 protons are consumed from the matrix,4 protons are released into the intermembrane space and 2 electrons arepassed to cytochrome c (By similarity). The 2 core subunits UQCRC1/QCR1and UQCRC2/QCR2 are homologous to the 2 mitochondrial-processingpeptidase (MPP) subunits beta-MPP and alpha-MPP respectively, and theyseem to have preserved their MPP processing properties (By similarity).May be involved in the in situ processing of UQCRFS1 into the matureRieske protein and its mitochondrial targeting sequence (MTS)/subunit 9when incorporated into complex III (Probable).{ECO:0000250|UniProtKB:P07257, ECO:0000250|UniProtKB:P23004,ECO:0000305|PubMed:29243944}.",
"references": []
},
{
"targ_id": "T004003",
"parent_targ_id": "T004",
"full_name": "Cytochrome b5 outer mitochondrial membrane isoform",
"abbrev": "Cytochrome b5 type B",
"protein_names": "Cytochrome b5 type B (Cytochrome b5 outer mitochondrial membrane isoform)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "O43169",
"Uniprot_name": "CYB5B_HUMAN",
"EC_numbers": "",
"gene_symbol": "CYB5B",
"gene_synonyms": "CYB5-M",
"gene_synonyms_links": "",
"gene_name": "Cytochrome b5 type B",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000103018",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion outer membrane",
"reaction": "",
"targ_desciption": "Cytochrome b5 is a membrane-bound hemoprotein functioning asan electron carrier for several membrane-bound oxygenases.{ECO:0000250}.",
"references": []
},
{
"targ_id": "T004004",
"parent_targ_id": "T004",
"full_name": "Cytochrome b-c1 complex subunit 7",
"abbrev": "Complex III subunit 7",
"protein_names": "Cytochrome b-c1 complex subunit 7 (Complex III subunit 7) (Complex III subunit VII) (QP-C) (Ubiquinol-cytochrome c reductase complex 14 kDa protein)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "P14927",
"Uniprot_name": "QCR7_HUMAN",
"EC_numbers": "",
"gene_symbol": "UQCRB",
"gene_synonyms": "QCR7, QP-C, UQBP, UQCR6",
"gene_synonyms_links": "",
"gene_name": "Ubiquinol-cytochrome c reductase binding protein",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000156467",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Peripheral membrane protein; Matrix side",
"reaction": "",
"targ_desciption": "Component of the ubiquinol-cytochrome c oxidoreductase, amultisubunit transmembrane complex that is part of the mitochondrialelectron transport chain which drives oxidative phosphorylation. Therespiratory chain contains 3 multisubunit complexes succinatedehydrogenase (complex II, CII), ubiquinol-cytochrome c oxidoreductase(cytochrome b-c1 complex, complex III, CIII) and cytochrome c oxidase(complex IV, CIV), that cooperate to transfer electrons derived fromNADH and succinate to molecular oxygen, creating an electrochemicalgradient over the inner membrane that drives transmembrane transportand the ATP synthase. The cytochrome b-c1 complex catalyzes electrontransfer from ubiquinol to cytochrome c, linking this redox reaction totranslocation of protons across the mitochondrial inner membrane, withprotons being carried across the membrane as hydrogens on the quinol.In the process called Q cycle, 2 protons are consumed from the matrix,4 protons are released into the intermembrane space and 2 electrons arepassed to cytochrome c. {ECO:0000250|UniProtKB:P00128}.",
"references": []
},
{
"targ_id": "T004005",
"parent_targ_id": "T004",
"full_name": "Cytochrome c1, heme protein, mitochondrial",
"abbrev": "Complex III subunit 4",
"protein_names": "Cytochrome c1, heme protein, mitochondrial (EC 7.1.1.8) (Complex III subunit 4) (Complex III subunit IV) (Cytochrome b-c1 complex subunit 4) (Ubiquinol-cytochrome-c reductase complex cytochrome c1 subunit) (Cytochrome c-1)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "Translocase",
"Uniport_ID": "P08574",
"Uniprot_name": "CY1_HUMAN",
"EC_numbers": "7.1.1.8",
"gene_symbol": "CYC1",
"gene_synonyms": "UQCR4",
"gene_synonyms_links": "",
"gene_name": "Cytochrome c1",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000179091",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Single-pass membrane protein",
"reaction": "2 [Fe(III)cytochrome c](out) + a quinol = 2 [Fe(II)cytochromec](out) + a quinone + 2 H(+)(out)",
"targ_desciption": "Component of the ubiquinol-cytochrome c oxidoreductase, amultisubunit transmembrane complex that is part of the mitochondrialelectron transport chain which drives oxidative phosphorylation. Therespiratory chain contains 3 multisubunit complexes succinatedehydrogenase (complex II, CII), ubiquinol-cytochrome c oxidoreductase(cytochrome b-c1 complex, complex III, CIII) and cytochrome c oxidase(complex IV, CIV), that cooperate to transfer electrons derived fromNADH and succinate to molecular oxygen, creating an electrochemicalgradient over the inner membrane that drives transmembrane transportand the ATP synthase. The cytochrome b-c1 complex catalyzes electrontransfer from ubiquinol to cytochrome c, linking this redox reaction totranslocation of protons across the mitochondrial inner membrane, withprotons being carried across the membrane as hydrogens on the quinol.In the process called Q cycle, 2 protons are consumed from the matrix,4 protons are released into the intermembrane space and 2 electrons arepassed to cytochrome c. Cytochrome c1 is a catalytic core subunitcontaining a c-type heme. It transfers electrons from the [2Fe-2S]iron-sulfur cluster of the Rieske protein to cytochrome c.{ECO:0000250|UniProtKB:P07143}.",
"references": []
},
{
"targ_id": "T004006",
"parent_targ_id": "T004",
"full_name": "Mitochondrial chaperone BCS1",
"abbrev": "h-BCS1",
"protein_names": "Mitochondrial chaperone BCS1 (h-BCS1) (BCS1-like protein)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "Chaperone",
"Uniport_ID": "Q9Y276",
"Uniprot_name": "BCS1_HUMAN",
"EC_numbers": "",
"gene_symbol": "BCS1L",
"gene_synonyms": "BCS, BJS, h-BCS, Hs.6719",
"gene_synonyms_links": "",
"gene_name": "BCS1 homolog, ubiquinol-cytochrome c reductase complex chaperone",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000074582",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Single-pass membrane protein",
"reaction": "",
"targ_desciption": "Chaperone necessary for the assembly of mitochondrialrespiratory chain complex III. Plays an important role in themaintenance of mitochondrial tubular networks, respiratory chainassembly and formation of the LETM1 complex.{ECO:0000269|PubMed:18628306}.",
"references": []
},
{
"targ_id": "T004007",
"parent_targ_id": "T004",
"full_name": "Mitogen-activated protein kinase kinase kinase 20",
"abbrev": "HCCS-4",
"protein_names": "Mitogen-activated protein kinase kinase kinase 20 (EC 2.7.11.25) (Human cervical cancer suppressor gene 4 protein) (HCCS-4) (Leucine zipper- and sterile alpha motif-containing kinase) (MLK-like mitogen-activated protein triple kinase) (Mitogen-activated protein kinase kinase kinase MLT) (Mixed lineage kinase-related kinase) (MLK-related kinase) (MRK) (Sterile alpha motif- and leucine zipper-containing kinase AZK)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "Q9NYL2",
"Uniprot_name": "M3K20_HUMAN",
"EC_numbers": "2.7.11.25",
"gene_symbol": "",
"gene_synonyms": "",
"gene_synonyms_links": "",
"gene_name": "",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "",
"omim_id": "",
"genecard_link": "",
"locs": "Cytoplasm; Nucleus",
"reaction": "ATP + L-seryl-[protein] = ADP + H(+) + O-phospho-L-seryl-[protein]; ATP + L-threonyl-[protein] = ADP + H(+) + O-phospho-L-threonyl-[protein]",
"targ_desciption": "Stress-activated component of a protein kinase signaltransduction cascade. Regulates the JNK and p38 pathways. Part of asignaling cascade that begins with the activation of the adrenergicreceptor ADRA1B and leads to the activation of MAPK14. Pro-apoptotic.Role in regulation of S and G2 cell cycle checkpoint by directphosphorylation of CHEK2 (PubMed:10924358, PubMed:11836244,PubMed:15342622, PubMed:21224381). Involved in limb development(PubMed:26755636). {ECO:0000269|PubMed:10924358,ECO:0000269|PubMed:11836244, ECO:0000269|PubMed:15342622,ECO:0000269|PubMed:21224381, ECO:0000269|PubMed:26755636}.; [Isoform 1]: Phosphorylates histone H3 at 'Ser-28'(PubMed:15684425). May have role in neoplastic cell transformation andcancer development (PubMed:15172994). Causes cell shrinkage anddisruption of actin stress fibers (PubMed:11042189).{ECO:0000269|PubMed:11042189, ECO:0000269|PubMed:15172994,ECO:0000269|PubMed:15684425}.",
"references": []
},
{
"targ_id": "T004008",
"parent_targ_id": "T004",
"full_name": "Tetratricopeptide repeat protein 19, mitochondrial",
"abbrev": "TPR repeat protein 19",
"protein_names": "Tetratricopeptide repeat protein 19, mitochondrial (TPR repeat protein 19)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "Q6DKK2",
"Uniprot_name": "TTC19_HUMAN",
"EC_numbers": "",
"gene_symbol": "TTC19",
"gene_synonyms": "FLJ20343, MGC19520",
"gene_synonyms_links": "",
"gene_name": "Tetratricopeptide repeat domain 19",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000011295",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane",
"reaction": "",
"targ_desciption": "Required for the preservation of the structural andfunctional integrity of mitochondrial respiratory complex III byallowing the physiological turnover of the Rieske protein UQCRFS1(PubMed:21278747, PubMed:28673544). Involved in the clearance ofUQCRFS1 N-terminal fragments, which are produced upon incorporation ofUQCRFS1 into the complex III and whose presence is detrimental for itscatalytic activity (PubMed:28673544). {ECO:0000269|PubMed:21278747,ECO:0000269|PubMed:28673544}.",
"references": []
},
{
"targ_id": "T004009",
"parent_targ_id": "T004",
"full_name": "Complex III assembly factor LYRM7",
"abbrev": "",
"protein_names": "Complex III assembly factor LYRM7 (LYR motif-containing protein 7)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "Chaperone",
"Uniport_ID": "Q5U5X0",
"Uniprot_name": "LYRM7_HUMAN",
"EC_numbers": "",
"gene_symbol": "LYRM7",
"gene_synonyms": "C5orf31, FLJ20796, MZM1L",
"gene_synonyms_links": "",
"gene_name": "LYR motif containing 7",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000186687",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion matrix",
"reaction": "",
"targ_desciption": "Assembly factor required for Rieske Fe-S protein UQCRFS1incorporation into the cytochrome b-c1 (CIII) complex. Functions as achaperone, binding to this subunit within the mitochondrial matrix andstabilizing it prior to its translocation and insertion into the lateCIII dimeric intermediate within the mitochondrial inner membrane.{ECO:0000269|PubMed:23168492}.",
"references": []
},
{
"targ_id": "T004010",
"parent_targ_id": "T004",
"full_name": "Ubiquinol-cytochrome-c reductase complex assembly factor 2",
"abbrev": "",
"protein_names": "Ubiquinol-cytochrome-c reductase complex assembly factor 2 (Breast cancer-associated protein SGA-81M) (Mitochondrial nucleoid factor 1) (Mitochondrial protein M19)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "Q9BRT2",
"Uniprot_name": "UQCC2_HUMAN",
"EC_numbers": "",
"gene_symbol": "UQCC2",
"gene_synonyms": "bA6B20.2, C6orf125, Cbp6, M19, MGC14833, MNF1",
"gene_synonyms_links": "",
"gene_name": "Ubiquinol-cytochrome c reductase complex assembly factor 2",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000137288",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion matrix, mitochondrion nucleoid; Mitochondrion; Mitochondrion intermembrane space; Mitochondrion matrix; Mitochondrion inner membrane",
"reaction": "",
"targ_desciption": "Required for the assembly of the ubiquinol-cytochrome creductase complex (mitochondrial respiratory chain complex III orcytochrome b-c1 complex). Plays a role in the modulation of respiratorychain activities such as oxygen consumption and ATP production and viaits modulation of the respiratory chain activity can regulate skeletalmuscle differentiation and insulin secretion by pancreatic beta-cells.Involved in cytochrome b translation and/or stability.{ECO:0000269|PubMed:22363741, ECO:0000269|PubMed:24385928}.",
"references": []
},
{
"targ_id": "T004011",
"parent_targ_id": "T004",
"full_name": "Ubiquinol-cytochrome-c reductase complex assembly factor 3",
"abbrev": "",
"protein_names": "Ubiquinol-cytochrome-c reductase complex assembly factor 3 (Assembly factor CBP4 homolog)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "Q6UW78",
"Uniprot_name": "UQCC3_HUMAN",
"EC_numbers": "",
"gene_symbol": "UQCC3",
"gene_synonyms": "C11orf83, UNQ655",
"gene_synonyms_links": "",
"gene_name": "Ubiquinol-cytochrome c reductase complex assembly factor 3",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000204922",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Single-pass membrane protein",
"reaction": "",
"targ_desciption": "Required for the assembly of the ubiquinol-cytochrome creductase complex (mitochondrial respiratory chain complex III orcytochrome b-c1 complex), mediating cytochrome b recruitment andprobably stabilization within the complex. Thereby, plays an importantrole in ATP production by mitochondria. Cardiolipin-binding protein, itmay also control the cardiolipin composition of mitochondria membranesand their morphology. {ECO:0000269|PubMed:25008109,ECO:0000269|PubMed:25605331}.",
"references": []
},
{
"targ_id": "T004012",
"parent_targ_id": "T004",
"full_name": "Qo site (Qp site or ubiquinol oxidation site)",
"abbrev": "Qo site (Qp site or ubiquinol oxidation site)",
"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": "class P inhibitors (both of Pm and Pf subtypes) can bind to Qo site",
"references": [
"RC01148",
"RC01149",
"RC01150",
"RC01151",
"RC01152",
"RC01153",
"RC01154",
"RC01155",
"RC01156",
"RC01157",
"RC01158",
"RC01159",
"RC01163",
"RC03453",
"RC03465"
]
},
{
"targ_id": "T004013",
"parent_targ_id": "T004",
"full_name": "Qi site (Qn site or quinone reduction site)",
"abbrev": "Qi site (Qn site or quinone reduction site)",
"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": "class N inhibitors can bind to Qo site",
"references": [
"RC01160",
"RC01161",
"RC01162"
]
},
{
"targ_id": "T004016",
"parent_targ_id": "T004",
"full_name": "Cytochrome b-c1 complex subunit Rieske, mitochondrial",
"abbrev": "Complex III subunit 5",
"protein_names": "Cytochrome b-c1 complex subunit Rieske, mitochondrial (EC 7.1.1.8) (Complex III subunit 5) (Cytochrome b-c1 complex subunit 5) (Rieske iron-sulfur protein) (RISP) (Rieske protein UQCRFS1) (Ubiquinol-cytochrome c reductase iron-sulfur subunit) [Cleaved into: Cytochrome b-c1 complex subunit 9 (Su9) (Subunit 9) (8 kDa subunit 9) (Complex III subunit IX) (Cytochrome b-c1 complex subunit 11) (UQCRFS1 mitochondrial targeting sequence) (UQCRFS1 MTS) (Ubiquinol-cytochrome c reductase 8 kDa protein)]",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "Translocase",
"Uniport_ID": "P47985",
"Uniprot_name": "UCRI_HUMAN",
"EC_numbers": "7.1.1.8",
"gene_symbol": "UQCRFS1",
"gene_synonyms": "RIP1, RIS1, RISP, UQCR5",
"gene_synonyms_links": "",
"gene_name": "Ubiquinol-cytochrome c reductase, Rieske iron-sulfur polypeptide 1",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000169021",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Single-pass membrane protein",
"reaction": "2 [Fe(III)cytochrome c](out) + a quinol = 2 [Fe(II)cytochromec](out) + a quinone + 2 H(+)(out)",
"targ_desciption": "[Cytochrome b-c1 complex subunit Rieske, mitochondrial]:Component of the ubiquinol-cytochrome c oxidoreductase, a multisubunittransmembrane complex that is part of the mitochondrial electrontransport chain which drives oxidative phosphorylation. The respiratorychain contains 3 multisubunit complexes 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 to transfer electrons derived from NADH andsuccinate to molecular oxygen, creating an electrochemical gradientover the inner membrane that drives transmembrane transport and the ATPsynthase. The cytochrome b-c1 complex catalyzes electron transfer fromubiquinol to cytochrome c, linking this redox reaction to translocationof protons across the mitochondrial inner membrane, with protons beingcarried across the membrane as hydrogens on the quinol. In the processcalled Q cycle, 2 protons are consumed from the matrix, 4 protons arereleased into the intermembrane space and 2 electrons are passed tocytochrome c. The Rieske protein is a catalytic core subunit containinga [2Fe-2S] iron-sulfur cluster. It cycles between 2 conformationalstates during catalysis to transfer electrons from the quinol bound inthe Q(0) site in cytochrome b to cytochrome c1 (By similarity).Incorporation of UQCRFS1 is the penultimate step in complex IIIassembly (PubMed:28673544). {ECO:0000250|UniProtKB:P08067,ECO:0000269|PubMed:28673544}.; [Cytochrome b-c1 complex subunit 9]: Component of theubiquinol-cytochrome c oxidoreductase (cytochrome b-c1 complex, complexIII, CIII). UQCRFS1 undergoes proteolytic processing once it isincorporated in the complex III dimer. One of the fragments, calledsubunit 9, corresponds to its mitochondrial targeting sequence (MTS).The proteolytic processing is necessary for the correct insertion ofUQCRFS1 in the complex III dimer, but the persistence of UQCRFS1-derived fragments may prevent newly imported UQCRFS1 to be processedand assembled into complex III and is detrimental for the complex IIIstructure and function. {ECO:0000269|PubMed:28673544}.",
"references": []
},
{
"targ_id": "T004017",
"parent_targ_id": "T004",
"full_name": "Cytochrome b-c1 complex subunit 8",
"abbrev": "Complex III subunit 8",
"protein_names": "Cytochrome b-c1 complex subunit 8 (Complex III subunit 8) (Complex III subunit VIII) (Ubiquinol-cytochrome c reductase complex 9.5 kDa protein) (Ubiquinol-cytochrome c reductase complex ubiquinone-binding protein QP-C)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "O14949",
"Uniprot_name": "QCR8_HUMAN",
"EC_numbers": "",
"gene_symbol": "UQCRQ",
"gene_synonyms": "QCR8, QP-C, UQCR7",
"gene_synonyms_links": "",
"gene_name": "Ubiquinol-cytochrome c reductase complex III subunit VII",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000164405",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Single-pass membrane protein",
"reaction": "",
"targ_desciption": "Component of the ubiquinol-cytochrome c oxidoreductase, amultisubunit transmembrane complex that is part of the mitochondrialelectron transport chain which drives oxidative phosphorylation. Therespiratory chain contains 3 multisubunit complexes succinatedehydrogenase (complex II, CII), ubiquinol-cytochrome c oxidoreductase(cytochrome b-c1 complex, complex III, CIII) and cytochrome c oxidase(complex IV, CIV), that cooperate to transfer electrons derived fromNADH and succinate to molecular oxygen, creating an electrochemicalgradient over the inner membrane that drives transmembrane transportand the ATP synthase. The cytochrome b-c1 complex catalyzes electrontransfer from ubiquinol to cytochrome c, linking this redox reaction totranslocation of protons across the mitochondrial inner membrane, withprotons being carried across the membrane as hydrogens on the quinol.In the process called Q cycle, 2 protons are consumed from the matrix,4 protons are released into the intermembrane space and 2 electrons arepassed to cytochrome c. {ECO:0000250|UniProtKB:P08525}.",
"references": []
},
{
"targ_id": "T004018",
"parent_targ_id": "T004",
"full_name": "Cytochrome b-c1 complex subunit 10",
"abbrev": "Complex III subunit 10",
"protein_names": "Cytochrome b-c1 complex subunit 10 (Complex III subunit 10) (Complex III subunit XI) (Ubiquinol-cytochrome c reductase complex 6.4 kDa protein)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "O14957",
"Uniprot_name": "QCR10_HUMAN",
"EC_numbers": "",
"gene_symbol": "UQCR11",
"gene_synonyms": "QCR10, UQCR",
"gene_synonyms_links": "",
"gene_name": "Ubiquinol-cytochrome c reductase, complex III subunit XI",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000127540",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Single-pass membrane protein",
"reaction": "",
"targ_desciption": "Component of the ubiquinol-cytochrome c oxidoreductase, amultisubunit transmembrane complex that is part of the mitochondrialelectron transport chain which drives oxidative phosphorylation. Therespiratory chain contains 3 multisubunit complexes succinatedehydrogenase (complex II, CII), ubiquinol-cytochrome c oxidoreductase(cytochrome b-c1 complex, complex III, CIII) and cytochrome c oxidase(complex IV, CIV), that cooperate to transfer electrons derived fromNADH and succinate to molecular oxygen, creating an electrochemicalgradient over the inner membrane that drives transmembrane transportand the ATP synthase. The cytochrome b-c1 complex catalyzes electrontransfer from ubiquinol to cytochrome c, linking this redox reaction totranslocation of protons across the mitochondrial inner membrane, withprotons being carried across the membrane as hydrogens on the quinol.In the process called Q cycle, 2 protons are consumed from the matrix,4 protons are released into the intermembrane space and 2 electrons arepassed to cytochrome c. QCR10 has a role in CIII assembly and RIP1stability. {ECO:0000250|UniProtKB:P37299}.",
"references": []
},
{
"targ_id": "T004019",
"parent_targ_id": "T004",
"full_name": "Cytochrome b-c1 complex subunit 9",
"abbrev": "Complex III subunit 9",
"protein_names": "Cytochrome b-c1 complex subunit 9 (Complex III subunit 9) (Complex III subunit X) (Cytochrome c1 non-heme 7 kDa protein) (Ubiquinol-cytochrome c reductase complex 7.2 kDa protein)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "Q9UDW1",
"Uniprot_name": "QCR9_HUMAN",
"EC_numbers": "",
"gene_symbol": "UQCR10",
"gene_synonyms": "HSPC051, QCR9, UCCR7.2, UCRC",
"gene_synonyms_links": "",
"gene_name": "Ubiquinol-cytochrome c reductase, complex III subunit X",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000184076",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Single-pass membrane protein",
"reaction": "",
"targ_desciption": "Component of the ubiquinol-cytochrome c oxidoreductase, amultisubunit transmembrane complex that is part of the mitochondrialelectron transport chain which drives oxidative phosphorylation. Therespiratory chain contains 3 multisubunit complexes succinatedehydrogenase (complex II, CII), ubiquinol-cytochrome c oxidoreductase(cytochrome b-c1 complex, complex III, CIII) and cytochrome c oxidase(complex IV, CIV), that cooperate to transfer electrons derived fromNADH and succinate to molecular oxygen, creating an electrochemicalgradient over the inner membrane that drives transmembrane transportand the ATP synthase. The cytochrome b-c1 complex catalyzes electrontransfer from ubiquinol to cytochrome c, linking this redox reaction totranslocation of protons across the mitochondrial inner membrane, withprotons being carried across the membrane as hydrogens on the quinol.In the process called Q cycle, 2 protons are consumed from the matrix,4 protons are released into the intermembrane space and 2 electrons arepassed to cytochrome c. {ECO:0000250|UniProtKB:P22289}.",
"references": []
},
{
"targ_id": "T004020",
"parent_targ_id": "T004",
"full_name": "Cytochrome b-c1 complex subunit 6, mitochondrial",
"abbrev": "Complex III subunit 6",
"protein_names": "Cytochrome b-c1 complex subunit 6, mitochondrial (Complex III subunit 6) (Complex III subunit VIII) (Cytochrome c1 non-heme 11 kDa protein) (Mitochondrial hinge protein) (Ubiquinol-cytochrome c reductase complex 11 kDa protein)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "P07919",
"Uniprot_name": "QCR6_HUMAN",
"EC_numbers": "",
"gene_symbol": "UQCRH",
"gene_synonyms": "QCR6, UQCR8",
"gene_synonyms_links": "",
"gene_name": "Ubiquinol-cytochrome c reductase hinge protein",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000173660",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Peripheral membrane protein; Intermembrane side",
"reaction": "",
"targ_desciption": "Component of the ubiquinol-cytochrome c oxidoreductase, amultisubunit transmembrane complex that is part of the mitochondrialelectron transport chain which drives oxidative phosphorylation. Therespiratory chain contains 3 multisubunit complexes succinatedehydrogenase (complex II, CII), ubiquinol-cytochrome c oxidoreductase(cytochrome b-c1 complex, complex III, CIII) and cytochrome c oxidase(complex IV, CIV), that cooperate to transfer electrons derived fromNADH and succinate to molecular oxygen, creating an electrochemicalgradient over the inner membrane that drives transmembrane transportand the ATP synthase. The cytochrome b-c1 complex catalyzes electrontransfer from ubiquinol to cytochrome c, linking this redox reaction totranslocation of protons across the mitochondrial inner membrane, withprotons being carried across the membrane as hydrogens on the quinol.In the process called Q cycle, 2 protons are consumed from the matrix,4 protons are released into the intermembrane space and 2 electrons arepassed to cytochrome c. {ECO:0000250|UniProtKB:P00127}.",
"references": []
},
{
"targ_id": "T004021",
"parent_targ_id": "T004",
"full_name": "Cytochrome b-c1 complex subunit 1, mitochondrial",
"abbrev": "Complex III subunit 1",
"protein_names": "Cytochrome b-c1 complex subunit 1, mitochondrial (Complex III subunit 1) (Core protein I) (Ubiquinol-cytochrome-c reductase complex core protein 1)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "",
"Uniport_ID": "P31930",
"Uniprot_name": "QCR1_HUMAN",
"EC_numbers": "",
"gene_symbol": "UQCRC1",
"gene_synonyms": "D3S3191, QCR1, UQCR1",
"gene_synonyms_links": "",
"gene_name": "Ubiquinol-cytochrome c reductase core protein 1",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000010256",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Peripheral membrane protein; Matrix side",
"reaction": "",
"targ_desciption": "Component of the ubiquinol-cytochrome c oxidoreductase, amultisubunit transmembrane complex that is part of the mitochondrialelectron transport chain which drives oxidative phosphorylation. Therespiratory chain contains 3 multisubunit complexes succinatedehydrogenase (complex II, CII), ubiquinol-cytochrome c oxidoreductase(cytochrome b-c1 complex, complex III, CIII) and cytochrome c oxidase(complex IV, CIV), that cooperate to transfer electrons derived fromNADH and succinate to molecular oxygen, creating an electrochemicalgradient over the inner membrane that drives transmembrane transportand the ATP synthase. The cytochrome b-c1 complex catalyzes electrontransfer from ubiquinol to cytochrome c, linking this redox reaction totranslocation of protons across the mitochondrial inner membrane, withprotons being carried across the membrane as hydrogens on the quinol.In the process called Q cycle, 2 protons are consumed from the matrix,4 protons are released into the intermembrane space and 2 electrons arepassed to cytochrome c (By similarity). The 2 core subunits UQCRC1/QCR1and UQCRC2/QCR2 are homologous to the 2 mitochondrial-processingpeptidase (MPP) subunits beta-MPP and alpha-MPP respectively, and theyseem to have preserved their MPP processing properties (By similarity).May be involved in the in situ processing of UQCRFS1 into the matureRieske protein and its mitochondrial targeting sequence (MTS)/subunit 9when incorporated into complex III (Probable).{ECO:0000250|UniProtKB:P07256, ECO:0000250|UniProtKB:P31800,ECO:0000305|PubMed:29243944}.",
"references": []
},
{
"targ_id": "T005",
"parent_targ_id": "T",
"full_name": "Cytochrome c oxidase",
"abbrev": "Complex IV",
"protein_names": "",
"related_func_ids": "F020103",
"category": "enzyme",
"subcategories": "",
"Uniport_ID": "",
"Uniprot_name": "",
"EC_numbers": "7.1.1.9",
"gene_symbol": "",
"gene_synonyms": "",
"gene_synonyms_links": "",
"gene_name": "",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "",
"omim_id": "",
"genecard_link": "",
"locs": "",
"reaction": "4 [Fe(II)cytochrome c] + 4 H(+) + O2 = 4 [Fe(III)cytochrome c]+ 2 H2O",
"targ_desciption": "",
"references": [
"RC00115",
"RC00120",
"RC00125",
"RC00130",
"RC00135",
"RC00140",
"RC00145",
"RC00150",
"RC00155",
"RC00160",
"RC00165",
"RC00170",
"RC00175",
"RC00180",
"RC00185",
"RC00190",
"RC00195",
"RC00200",
"RC00205",
"RC00210",
"RC00215",
"RC00925",
"RC00956",
"RC01170",
"RC01171",
"RC01172",
"RC01173",
"RC03716",
"RC03920",
"RC04709",
"RC04710",
"RC04711",
"RC04712",
"RC04731",
"RC04763",
"RC04764",
"RC04956"
]
},
{
"targ_id": "T005001",
"parent_targ_id": "T005",
"full_name": "Cytochrome c oxidase subunit 1",
"abbrev": "Cytochrome c oxidase subunit 1",
"protein_names": "Cytochrome c oxidase subunit 1 (EC 7.1.1.9) (Cytochrome c oxidase polypeptide I)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "Translocase",
"Uniport_ID": "P00395",
"Uniprot_name": "COX1_HUMAN",
"EC_numbers": "7.1.1.9",
"gene_symbol": "MT-CO1",
"gene_synonyms": "COI, COX1, MTCO1",
"gene_synonyms_links": "",
"gene_name": "Mitochondrially encoded cytochrome c oxidase I",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000198804",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Multi-pass membrane protein",
"reaction": "4 [Fe(II)cytochrome c] + 4 H(+) + O2 = 4 [Fe(III)cytochrome c]+ 2 H2O",
"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:P00401}.",
"references": []
},
{
"targ_id": "T005002",
"parent_targ_id": "T005",
"full_name": "Cytochrome c oxidase subunit 2",
"abbrev": "Cytochrome c oxidase subunit 2",
"protein_names": "Cytochrome c oxidase subunit 2 (EC 7.1.1.9) (Cytochrome c oxidase polypeptide II)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "Translocase",
"Uniport_ID": "P00403",
"Uniprot_name": "COX2_HUMAN",
"EC_numbers": "7.1.1.9",
"gene_symbol": "MT-CO2",
"gene_synonyms": "CO2, COX2, MTCO2",
"gene_synonyms_links": "",
"gene_name": "Mitochondrially encoded cytochrome c oxidase II",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000198712",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Multi-pass membrane protein",
"reaction": "4 [Fe(II)cytochrome c] + 4 H(+) + O2 = 4 [Fe(III)cytochrome c]+ 2 H2O",
"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:P00410}.",
"references": []
},
{
"targ_id": "T005003",
"parent_targ_id": "T005",
"full_name": "Cytochrome c oxidase subunit 3",
"abbrev": "Cytochrome c oxidase subunit 3",
"protein_names": "Cytochrome c oxidase subunit 3 (EC 7.1.1.9) (Cytochrome c oxidase polypeptide III)",
"related_func_ids": "F020103",
"category": "protein subunit",
"subcategories": "Translocase",
"Uniport_ID": "P00414",
"Uniprot_name": "COX3_HUMAN",
"EC_numbers": "7.1.1.9",
"gene_symbol": "MT-CO3",
"gene_synonyms": "CO3, COIII, COX3, MTCO3",
"gene_synonyms_links": "",
"gene_name": "Mitochondrially encoded cytochrome c oxidase III",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000198938",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Multi-pass membrane protein",
"reaction": "4 [Fe(II)cytochrome c] + 4 H(+) + O2 = 4 [Fe(III)cytochrome c]+ 2 H2O",
"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:P00420}.",
"references": []
}
]
}
