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{
"count": 876,
"next": "https://mitotox.org/api/targets/list?format=api&page=8",
"previous": "https://mitotox.org/api/targets/list?format=api&page=6",
"results": [
{
"targ_id": "T438",
"parent_targ_id": "T",
"full_name": "Glycine amidinotransferase, mitochondrial",
"abbrev": "Transamidinase",
"protein_names": "Glycine amidinotransferase, mitochondrial (EC 2.1.4.1) (L-arginine:glycine amidinotransferase) (Transamidinase)",
"related_func_ids": "F0218",
"category": "enzyme",
"subcategories": "Transferase",
"Uniport_ID": "P50440",
"Uniprot_name": "GATM_HUMAN",
"EC_numbers": "2.1.4.1",
"gene_symbol": "GATM",
"gene_synonyms": "AGAT",
"gene_synonyms_links": "",
"gene_name": "Glycine amidinotransferase",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000171766",
"omim_id": "",
"genecard_link": "",
"locs": "[Isoform 1]: Mitochondrion inner membrane; Peripheral membrane protein; Intermembrane side; [Isoform 2]: Cytoplasm",
"reaction": "glycine + L-arginine = guanidinoacetate + L-ornithine",
"targ_desciption": "Catalyzes the biosynthesis of guanidinoacetate, the immediateprecursor of creatine. Creatine plays a vital role in energy metabolismin muscle tissues. May play a role in embryonic and central nervoussystem development. May be involved in the response to heart failure byelevating local creatine synthesis. {ECO:0000269|PubMed:16125225,ECO:0000269|PubMed:16614068, ECO:0000269|PubMed:16820567}.",
"references": []
},
{
"targ_id": "T439",
"parent_targ_id": "T",
"full_name": "Glutamate dehydrogenase 2, mitochondrial",
"abbrev": "GDH 2",
"protein_names": "Glutamate dehydrogenase 2, mitochondrial (GDH 2) (EC 1.4.1.3)",
"related_func_ids": "F020706; F020707; F060301",
"category": "enzyme",
"subcategories": "Oxidoreductase",
"Uniport_ID": "P49448",
"Uniprot_name": "DHE4_HUMAN",
"EC_numbers": "1.4.1.3",
"gene_symbol": "GLUD2",
"gene_synonyms": "GLUDP1",
"gene_synonyms_links": "",
"gene_name": "Glutamate dehydrogenase 2",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000182890",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion matrix",
"reaction": "H2O + L-glutamate + NAD(+) = 2-oxoglutarate + H(+) + NADH +NH4(+); H2O + L-glutamate + NADP(+) = 2-oxoglutarate + H(+) + NADPH +NH4(+)",
"targ_desciption": "Important for recycling the chief excitatoryneurotransmitter, glutamate, during neurotransmission.",
"references": []
},
{
"targ_id": "T440",
"parent_targ_id": "T",
"full_name": "Glycine N-acyltransferase-like protein 1",
"abbrev": "Glutamine N-acyltransferase",
"protein_names": "Glycine N-acyltransferase-like protein 1 (EC 2.3.1.68) (Acyl-CoA:glycine N-acyltransferase-like protein 1) (Glutamine N-acyltransferase)",
"related_func_ids": "F020706",
"category": "enzyme",
"subcategories": "Acyltransferase, Transferase",
"Uniport_ID": "Q969I3",
"Uniprot_name": "GLYL1_HUMAN",
"EC_numbers": "2.3.1.68",
"gene_symbol": "GLYATL1 GNAT",
"gene_synonyms": "",
"gene_synonyms_links": "",
"gene_name": "",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "",
"omim_id": "",
"genecard_link": "",
"locs": "",
"reaction": "an acyl-CoA + L-glutamine = an N(2)-acyl-L-glutamine + CoA +H(+)",
"targ_desciption": "Acyltransferase which transfers an acyl group to the N-terminus of glutamine. Can use phenylacetyl-CoA as an acyl donor.{ECO:0000269|PubMed:22475485}.",
"references": []
},
{
"targ_id": "T441",
"parent_targ_id": "T",
"full_name": "Proline dehydrogenase 1, mitochondrial",
"abbrev": "Proline oxidase",
"protein_names": "Proline dehydrogenase 1, mitochondrial (EC 1.5.5.2) (Proline oxidase) (Proline oxidase 2) (p53-induced gene 6 protein)",
"related_func_ids": "F020715",
"category": "enzyme",
"subcategories": "Oxidoreductase",
"Uniport_ID": "O43272",
"Uniprot_name": "PROD_HUMAN",
"EC_numbers": "1.5.5.2",
"gene_symbol": "PRODH",
"gene_synonyms": "HSPOX2, PIG6, PRODH1, PRODH2, TP53I6",
"gene_synonyms_links": "",
"gene_name": "Proline dehydrogenase 1",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000100033",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion matrix",
"reaction": "a quinone + L-proline = (S)-1-pyrroline-5-carboxylate + aquinol + H(+)",
"targ_desciption": "Converts proline to delta-1-pyrroline-5-carboxylate.{ECO:0000269|PubMed:15662599}.",
"references": []
},
{
"targ_id": "T442",
"parent_targ_id": "T",
"full_name": "Serine hydroxymethyltransferase, mitochondrial",
"abbrev": "SHMT",
"protein_names": "Serine hydroxymethyltransferase, mitochondrial (SHMT) (EC 2.1.2.1) (Glycine hydroxymethyltransferase) (Serine methylase)",
"related_func_ids": "F020716",
"category": "enzyme",
"subcategories": "Transferase",
"Uniport_ID": "P34897",
"Uniprot_name": "GLYM_HUMAN",
"EC_numbers": "2.1.2.1",
"gene_symbol": "SHMT2",
"gene_synonyms": "SHMT",
"gene_synonyms_links": "",
"gene_name": "Serine hydroxymethyltransferase 2",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000182199",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion; Mitochondrion matrix, mitochondrion nucleoid; Mitochondrion inner membrane; Cytoplasm; Nucleus",
"reaction": "(6R)-5,10-methylene-5,6,7,8-tetrahydrofolate + glycine + H2O =(6S)-5,6,7,8-tetrahydrofolate + L-serine",
"targ_desciption": "Catalyzes the cleavage of serine to glycine accompanied withthe production of 5,10-methylenetetrahydrofolate, an essentialintermediate for purine biosynthesis (PubMed:24075985, PubMed:29364879,PubMed:25619277). Serine provides the major source of folate one-carbonin cells by catalyzing the transfer of one carbon from serine totetrahydrofolate (PubMed:25619277). Contributes to the de novomitochondrial thymidylate biosynthesis pathway via its role in glycineand tetrahydrofolate metabolism: thymidylate biosynthesis is requiredto prevent uracil accumulation in mtDNA (PubMed:21876188). Alsorequired for mitochondrial translation by producing 5,10-methylenetetrahydrofolate. 5,10-methylenetetrahydrofolate providingmethyl donors to produce the taurinomethyluridine base at the wobbleposition of some mitochondrial tRNAs (PubMed:29452640,PubMed:29364879). Associates with mitochondrial DNA (PubMed:18063578).In addition to its role in mitochondria, also plays a role in thedeubiquitination of target proteins as component of the BRISC complex:required for IFNAR1 deubiquitination by the BRISC complex(PubMed:24075985). {ECO:0000269|PubMed:18063578,ECO:0000269|PubMed:21876188, ECO:0000269|PubMed:24075985,ECO:0000269|PubMed:25619277, ECO:0000269|PubMed:29364879,ECO:0000269|PubMed:29452640}.",
"references": []
},
{
"targ_id": "T443",
"parent_targ_id": "T",
"full_name": "2-amino-3-ketobutyrate coenzyme A ligase, mitochondrial",
"abbrev": "AKB ligase",
"protein_names": "2-amino-3-ketobutyrate coenzyme A ligase, mitochondrial (AKB ligase) (EC 2.3.1.29) (Aminoacetone synthase) (Glycine acetyltransferase)",
"related_func_ids": "F020717",
"category": "enzyme",
"subcategories": "Acyltransferase, Transferase",
"Uniport_ID": "O75600",
"Uniprot_name": "KBL_HUMAN",
"EC_numbers": "2.3.1.29",
"gene_symbol": "GCAT",
"gene_synonyms": "KBL",
"gene_synonyms_links": "",
"gene_name": "Glycine C-acetyltransferase",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000100116",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion; Nucleus",
"reaction": "acetyl-CoA + glycine = (2S)-2-amino-3-oxobutanoate + CoA",
"targ_desciption": "",
"references": []
},
{
"targ_id": "T444",
"parent_targ_id": "T",
"full_name": "Aminomethyltransferase, mitochondrial",
"abbrev": "GCVT",
"protein_names": "Aminomethyltransferase, mitochondrial (EC 2.1.2.10) (Glycine cleavage system T protein) (GCVT)",
"related_func_ids": "F020716; F020708; F020717",
"category": "enzyme",
"subcategories": "Aminotransferase, Transferase",
"Uniport_ID": "P48728",
"Uniprot_name": "GCST_HUMAN",
"EC_numbers": "2.1.2.10",
"gene_symbol": "AMT",
"gene_synonyms": "GCST, NKH",
"gene_synonyms_links": "",
"gene_name": "Aminomethyltransferase",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000145020",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion",
"reaction": "(6S)-5,6,7,8-tetrahydrofolate + (R)-N(6)-(S(8)-aminomethyldihydrolipoyl)-L-lysyl-[protein] = (6R)-5,10-methylene-5,6,7,8-tetrahydrofolate + (R)-N(6)-dihydrolipoyl-L-lysyl-[protein] +NH4(+)",
"targ_desciption": "The glycine cleavage system catalyzes the degradation ofglycine. {ECO:0000269|PubMed:16051266}.",
"references": []
},
{
"targ_id": "T445",
"parent_targ_id": "T",
"full_name": "Glycine dehydrogenase",
"abbrev": "Glycine decarboxylase",
"protein_names": "Glycine dehydrogenase (decarboxylating), mitochondrial (EC 1.4.4.2) (Glycine cleavage system P protein) (Glycine decarboxylase) (Glycine dehydrogenase (aminomethyl-transferring))",
"related_func_ids": "F020708",
"category": "enzyme",
"subcategories": "Oxidoreductase",
"Uniport_ID": "P23378",
"Uniprot_name": "GCSP_HUMAN",
"EC_numbers": "1.4.4.2",
"gene_symbol": "GLDC",
"gene_synonyms": "GCSP, NKH",
"gene_synonyms_links": "",
"gene_name": "Glycine decarboxylase",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000178445",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion",
"reaction": "glycine + H(+) + N(6)-lipoyl-L-lysyl-[glycine-cleavage complexH protein] = (R)-N(6)-(S(8)-aminomethyldihydrolipoyl)-L-lysyl-[glycine-cleavage complex H protein] + CO2",
"targ_desciption": "The glycine cleavage system catalyzes the degradation ofglycine. The P protein (GLDC) binds the alpha-amino group of glycinethrough its pyridoxal phosphate cofactor. CO(2) is released and theremaining methylamine moiety is then transferred to the lipoamidecofactor of the H protein (GCSH). {ECO:0000269|PubMed:1993704,ECO:0000269|PubMed:1996985, ECO:0000269|PubMed:28244183}.",
"references": []
},
{
"targ_id": "T446",
"parent_targ_id": "T",
"full_name": "Branched-chain-amino-acid aminotransferase, mitochondrial",
"abbrev": "BCAT(m)",
"protein_names": "Branched-chain-amino-acid aminotransferase, mitochondrial (BCAT(m)) (EC 2.6.1.42) (Placental protein 18) (PP18)",
"related_func_ids": "F020710; F020711; F020720",
"category": "enzyme",
"subcategories": "Aminotransferase, Transferase",
"Uniport_ID": "O15382",
"Uniprot_name": "BCAT2_HUMAN",
"EC_numbers": "2.6.1.42",
"gene_symbol": "BCAT2",
"gene_synonyms": "BCAM, BCT2",
"gene_synonyms_links": "",
"gene_name": "Branched chain amino acid transaminase 2",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000105552",
"omim_id": "",
"genecard_link": "",
"locs": "[Isoform A]: Mitochondrion; [Isoform B]: Cytoplasm",
"reaction": "2-oxoglutarate + L-leucine = 4-methyl-2-oxopentanoate + L-glutamate; 2-oxoglutarate + L-isoleucine = (S)-3-methyl-2-oxopentanoate +L-glutamate; 2-oxoglutarate + L-valine = 3-methyl-2-oxobutanoate + L-glutamate",
"targ_desciption": "Catalyzes the first reaction in the catabolism of theessential branched chain amino acids leucine, isoleucine, and valine.May also function as a transporter of branched chain alpha-keto acids.",
"references": []
},
{
"targ_id": "T447",
"parent_targ_id": "T",
"full_name": "[3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial",
"abbrev": "BCKD-kinase",
"protein_names": "[3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial (EC 2.7.11.4) (Branched-chain alpha-ketoacid dehydrogenase kinase) (BCKD-kinase) (BCKDHKIN)",
"related_func_ids": "F020710; F020711; F020720",
"category": "enzyme",
"subcategories": "Kinase, Transferase",
"Uniport_ID": "O14874",
"Uniprot_name": "BCKD_HUMAN",
"EC_numbers": "2.7.11.4",
"gene_symbol": "BCKDK",
"gene_synonyms": "",
"gene_synonyms_links": "",
"gene_name": "Branched chain ketoacid dehydrogenase kinase",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000103507",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion matrix; Mitochondrion",
"reaction": "[3-methyl-2-oxobutanoate dehydrogenase]-L-serine + ATP = [3-methyl-2-oxobutanoate dehydrogenase]-O-phospho-L-serine + ADP + H(+)",
"targ_desciption": "Catalyzes the phosphorylation and inactivation of thebranched-chain alpha-ketoacid dehydrogenase complex, the key regulatoryenzyme of the valine, leucine and isoleucine catabolic pathways. Keyenzyme that regulate the activity state of the BCKD complex.{ECO:0000269|PubMed:24449431}.",
"references": []
},
{
"targ_id": "T448",
"parent_targ_id": "T",
"full_name": "Isobutyryl-CoA dehydrogenase, mitochondrial",
"abbrev": "IBDH",
"protein_names": "Isobutyryl-CoA dehydrogenase, mitochondrial (IBDH) (EC 1.3.8.-) (Activator-recruited cofactor 42 kDa component) (ARC42) (Acyl-CoA dehydrogenase family member 8) (ACAD-8)",
"related_func_ids": "F020720; F0206",
"category": "enzyme",
"subcategories": "Activator, Oxidoreductase",
"Uniport_ID": "Q9UKU7",
"Uniprot_name": "ACAD8_HUMAN",
"EC_numbers": "1.3.8.-",
"gene_symbol": "ACAD8",
"gene_synonyms": "",
"gene_synonyms_links": "",
"gene_name": "Acyl-CoA dehydrogenase family member 8",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000151498",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion",
"reaction": "2-methylpropanoyl-CoA + H(+) + oxidized [electron-transferflavoprotein] = (2E)-2-methylpropenoyl-CoA + reduced [electron-transfer flavoprotein]; (2S)-2-methylbutanoyl-CoA + H(+) + oxidized [electron-transferflavoprotein] = (2E)-2-methylbutenoyl-CoA + reduced [electron-transfer flavoprotein]; H(+) + oxidized [electron-transfer flavoprotein] + propanoyl-CoA = acryloyl-CoA + reduced [electron-transfer flavoprotein]",
"targ_desciption": "Isobutyryl-CoA dehydrogenase which catalyzes one of the stepsof the valine catabolic pathway (PubMed:11013134, PubMed:12359132,PubMed:16857760). To a lesser extent, is also able to catalyze theoxidation of (2S)-2-methylbutanoyl-CoA (PubMed:11013134,PubMed:12359132). {ECO:0000269|PubMed:11013134,ECO:0000269|PubMed:12359132, ECO:0000269|PubMed:16857760}.",
"references": []
},
{
"targ_id": "T449",
"parent_targ_id": "T",
"full_name": "3-hydroxyisobutyrate dehydrogenase, mitochondrial",
"abbrev": "HIBADH",
"protein_names": "3-hydroxyisobutyrate dehydrogenase, mitochondrial (HIBADH) (EC 1.1.1.31)",
"related_func_ids": "F020720",
"category": "enzyme",
"subcategories": "Oxidoreductase",
"Uniport_ID": "P31937",
"Uniprot_name": "3HIDH_HUMAN",
"EC_numbers": "1.1.1.31",
"gene_symbol": "HIBADH",
"gene_synonyms": "NS5ATP1",
"gene_synonyms_links": "",
"gene_name": "3-hydroxyisobutyrate dehydrogenase",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000106049",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion",
"reaction": "3-hydroxy-2-methylpropanoate + NAD(+) = 2-methyl-3-oxopropanoate + H(+) + NADH",
"targ_desciption": "",
"references": []
},
{
"targ_id": "T450",
"parent_targ_id": "T",
"full_name": "Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial",
"abbrev": "MMSDH",
"protein_names": "Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial (MMSDH) (Malonate-semialdehyde dehydrogenase [acylating]) (EC 1.2.1.18) (EC 1.2.1.27) (Aldehyde dehydrogenase family 6 member A1)",
"related_func_ids": "F020720",
"category": "enzyme",
"subcategories": "Oxidoreductase",
"Uniport_ID": "Q02252",
"Uniprot_name": "MMSA_HUMAN",
"EC_numbers": "1.2.1.18; 1.2.1.27",
"gene_symbol": "ALDH6A1",
"gene_synonyms": "MMSDH",
"gene_synonyms_links": "",
"gene_name": "Aldehyde dehydrogenase 6 family member A1",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000119711",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion",
"reaction": "2-methyl-3-oxopropanoate + CoA + H2O + NAD(+) = H(+) +hydrogencarbonate + NADH + propanoyl-CoA; 3-oxopropanoate + CoA + NAD(+) = acetyl-CoA + CO2 + NADH; 3-oxopropanoate + CoA + NADP(+) = acetyl-CoA + CO2 + NADPH",
"targ_desciption": "Plays a role in valine and pyrimidine metabolism. Binds fattyacyl-CoA.",
"references": []
},
{
"targ_id": "T451",
"parent_targ_id": "T",
"full_name": "Kynurenine/alpha-aminoadipate aminotransferase, mitochondrial",
"abbrev": "AadAT",
"protein_names": "Kynurenine/alpha-aminoadipate aminotransferase, mitochondrial (KAT/AadAT) (2-aminoadipate aminotransferase) (2-aminoadipate transaminase) (EC 2.6.1.39) (Alpha-aminoadipate aminotransferase) (AadAT) (Kynurenine aminotransferase II) (Kynurenine--oxoglutarate aminotransferase II) (Kynurenine--oxoglutarate transaminase 2) (EC 2.6.1.7) (Kynurenine--oxoglutarate transaminase II)",
"related_func_ids": "F020712; F020718",
"category": "enzyme",
"subcategories": "Aminotransferase, Transferase",
"Uniport_ID": "Q8N5Z0",
"Uniprot_name": "AADAT_HUMAN",
"EC_numbers": "2.6.1.39; 2.6.1.7",
"gene_symbol": "AADAT",
"gene_synonyms": "KAT2, KATII, KYAT2",
"gene_synonyms_links": "",
"gene_name": "Aminoadipate aminotransferase",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000109576",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion",
"reaction": "2-oxoglutarate + L-kynurenine = 4-(2-aminophenyl)-2,4-dioxobutanoate + L-glutamate; 2-oxoglutarate + L-2-aminoadipate = 2-oxoadipate + L-glutamate",
"targ_desciption": "Transaminase with broad substrate specificity. Hastransaminase activity towards aminoadipate, kynurenine, methionine andglutamate. Shows activity also towards tryptophan, aspartate andhydroxykynurenine. Accepts a variety of oxo-acids as amino-groupacceptors, with a preference for 2-oxoglutarate, 2-oxocaproic acid,phenylpyruvate and alpha-oxo-gamma-methiol butyric acid. Can also useglyoxylate as amino-group acceptor (in vitro).{ECO:0000269|PubMed:18620547}.",
"references": []
},
{
"targ_id": "T452",
"parent_targ_id": "T",
"full_name": "Alpha-aminoadipic semialdehyde synthase, mitochondrial",
"abbrev": "LKR/SDH",
"protein_names": "Alpha-aminoadipic semialdehyde synthase, mitochondrial (LKR/SDH) [Includes: Lysine ketoglutarate reductase (LKR) (LOR) (EC 1.5.1.8); Saccharopine dehydrogenase (SDH) (EC 1.5.1.9)]",
"related_func_ids": "F020712",
"category": "enzyme",
"subcategories": "Multifunctional enzyme, Oxidoreductase",
"Uniport_ID": "Q9UDR5",
"Uniprot_name": "AASS_HUMAN",
"EC_numbers": "1.5.1.8; 1.5.1.9",
"gene_symbol": "AASS",
"gene_synonyms": "LKRSDH, LORSDH",
"gene_synonyms_links": "",
"gene_name": "Aminoadipate-semialdehyde synthase",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000008311",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion",
"reaction": "H2O + L-saccharopine + NADP(+) = 2-oxoglutarate + H(+) + L-lysine + NADPH; H2O + L-saccharopine + NAD(+) = (S)-2-amino-6-oxohexanoate +H(+) + L-glutamate + NADH",
"targ_desciption": "Bifunctional enzyme that catalyzes the first two steps inlysine degradation. The N-terminal and the C-terminal contain lysine-ketoglutarate reductase and saccharopine dehydrogenase activity,respectively.",
"references": []
},
{
"targ_id": "T453",
"parent_targ_id": "T",
"full_name": "5-phosphohydroxy-L-lysine phospho-lyase",
"abbrev": "Alanine--glyoxylate aminotransferase 2-like 2",
"protein_names": "5-phosphohydroxy-L-lysine phospho-lyase (EC 4.2.3.134) (Alanine--glyoxylate aminotransferase 2-like 2)",
"related_func_ids": "F020712",
"category": "enzyme",
"subcategories": "Lyase",
"Uniport_ID": "Q8IUZ5",
"Uniprot_name": "AT2L2_HUMAN",
"EC_numbers": "4.2.3.134",
"gene_symbol": "PHYKPL",
"gene_synonyms": "AGXT2L2, MGC15875",
"gene_synonyms_links": "",
"gene_name": "5-phosphohydroxy-L-lysine phospho-lyase",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000175309",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion",
"reaction": "(5R)-5-phosphooxy-L-lysine + H2O = (S)-2-amino-6-oxohexanoate+ NH4(+) + phosphate",
"targ_desciption": "Catalyzes the pyridoxal-phosphate-dependent breakdown of 5-phosphohydroxy-L-lysine, converting it to ammonia, inorganic phosphateand 2-aminoadipate semialdehyde. {ECO:0000269|PubMed:22241472}.",
"references": []
},
{
"targ_id": "T454",
"parent_targ_id": "T",
"full_name": "Glutamate dehydrogenase 1, mitochondrial",
"abbrev": "GDH 1",
"protein_names": "Glutamate dehydrogenase 1, mitochondrial (GDH 1) (EC 1.4.1.3)",
"related_func_ids": "F020706; F020707",
"category": "enzyme",
"subcategories": "Oxidoreductase",
"Uniport_ID": "P00367",
"Uniprot_name": "DHE3_HUMAN",
"EC_numbers": "1.4.1.3",
"gene_symbol": "GLUD1",
"gene_synonyms": "GDH, GLUD",
"gene_synonyms_links": "",
"gene_name": "Glutamate dehydrogenase 1",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000148672",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion matrix",
"reaction": "H2O + L-glutamate + NAD(+) = 2-oxoglutarate + H(+) + NADH +NH4(+); H2O + L-glutamate + NADP(+) = 2-oxoglutarate + H(+) + NADPH +NH4(+)",
"targ_desciption": "Mitochondrial glutamate dehydrogenase that converts L-glutamate into alpha-ketoglutarate. Plays a key role in glutamineanaplerosis by producing alpha-ketoglutarate, an important intermediatein the tricarboxylic acid cycle. May be involved in learning and memoryreactions by increasing the turnover of the excitatory neurotransmitterglutamate (By similarity). {ECO:0000250}.",
"references": []
},
{
"targ_id": "T455",
"parent_targ_id": "T",
"full_name": "Ornithine aminotransferase, mitochondrial",
"abbrev": "Ornithine delta-aminotransferase",
"protein_names": "Ornithine aminotransferase, mitochondrial (EC 2.6.1.13) (Ornithine delta-aminotransferase) (Ornithine--oxo-acid aminotransferase) [Cleaved into: Ornithine aminotransferase, hepatic form; Ornithine aminotransferase, renal form]",
"related_func_ids": "F020706; F020707",
"category": "enzyme",
"subcategories": "Aminotransferase, Transferase",
"Uniport_ID": "P04181",
"Uniprot_name": "OAT_HUMAN",
"EC_numbers": "2.6.1.13",
"gene_symbol": "OAT",
"gene_synonyms": "HOGA",
"gene_synonyms_links": "",
"gene_name": "Ornithine aminotransferase",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000065154",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion matrix",
"reaction": "a 2-oxocarboxylate + L-ornithine = an L-alpha-amino acid + L-glutamate 5-semialdehyde",
"targ_desciption": "",
"references": []
},
{
"targ_id": "T456",
"parent_targ_id": "T",
"full_name": "Glutaminase liver isoform, mitochondrial",
"abbrev": "GLS",
"protein_names": "Glutaminase liver isoform, mitochondrial (GLS) (EC 3.5.1.2) (L-glutaminase) (L-glutamine amidohydrolase)",
"related_func_ids": "F020707; F0505",
"category": "enzyme",
"subcategories": "Hydrolase",
"Uniport_ID": "Q9UI32",
"Uniprot_name": "GLSL_HUMAN",
"EC_numbers": "3.5.1.2",
"gene_symbol": "GLS2",
"gene_synonyms": "GA, GLS, hLGA, LGA",
"gene_synonyms_links": "",
"gene_name": "Glutaminase 2",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000135423",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion",
"reaction": "H2O + L-glutamine = L-glutamate + NH4(+)",
"targ_desciption": "Plays an important role in the regulation of glutaminecatabolism. Promotes mitochondrial respiration and increases ATPgeneration in cells by catalyzing the synthesis of glutamate and alpha-ketoglutarate. Increases cellular anti-oxidant function via NADH andglutathione production. May play a role in preventing tumorproliferation. {ECO:0000269|PubMed:20378837}.",
"references": []
},
{
"targ_id": "T457",
"parent_targ_id": "T",
"full_name": "Ornithine carbamoyltransferase, mitochondrial",
"abbrev": "OTCase",
"protein_names": "Ornithine carbamoyltransferase, mitochondrial (EC 2.1.3.3) (Ornithine transcarbamylase) (OTCase)",
"related_func_ids": "F0223; F020702",
"category": "enzyme",
"subcategories": "Transferase",
"Uniport_ID": "P00480",
"Uniprot_name": "OTC_HUMAN",
"EC_numbers": "2.1.3.3",
"gene_symbol": "OTC",
"gene_synonyms": "",
"gene_synonyms_links": "",
"gene_name": "Ornithine carbamoyltransferase",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000036473",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion matrix",
"reaction": "carbamoyl phosphate + L-ornithine = H(+) + L-citrulline +phosphate",
"targ_desciption": "",
"references": []
},
{
"targ_id": "T458",
"parent_targ_id": "T",
"full_name": "Succinate-semialdehyde dehydrogenase, mitochondrial",
"abbrev": "Aldehyde dehydrogenase family 5 member A1",
"protein_names": "Succinate-semialdehyde dehydrogenase, mitochondrial (EC 1.2.1.24) (Aldehyde dehydrogenase family 5 member A1) (NAD(+)-dependent succinic semialdehyde dehydrogenase)",
"related_func_ids": "F020707",
"category": "enzyme",
"subcategories": "Oxidoreductase",
"Uniport_ID": "P51649",
"Uniprot_name": "SSDH_HUMAN",
"EC_numbers": "1.2.1.24",
"gene_symbol": "ALDH5A1",
"gene_synonyms": "SSADH, SSDH",
"gene_synonyms_links": "",
"gene_name": "Aldehyde dehydrogenase 5 family member A1",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000112294",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion",
"reaction": "H2O + NAD(+) + succinate semialdehyde = 2 H(+) + NADH +succinate",
"targ_desciption": "Catalyzes one step in the degradation of the inhibitoryneurotransmitter gamma-aminobutyric acid (GABA).{ECO:0000269|PubMed:19300440}.",
"references": []
},
{
"targ_id": "T459",
"parent_targ_id": "T",
"full_name": "Amine oxidase [flavin-containing] B",
"abbrev": "MAO-B",
"protein_names": "Amine oxidase [flavin-containing] B (EC 1.4.3.4) (Monoamine oxidase type B) (MAO-B)",
"related_func_ids": "F050401",
"category": "enzyme",
"subcategories": "Oxidoreductase",
"Uniport_ID": "P27338",
"Uniprot_name": "AOFB_HUMAN",
"EC_numbers": "1.4.3.4",
"gene_symbol": "MAOB",
"gene_synonyms": "",
"gene_synonyms_links": "",
"gene_name": "Monoamine oxidase B",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000069535",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion outer membrane; Single-pass type IV membrane protein; Cytoplasmic side",
"reaction": "a secondary aliphatic amine + H2O + O2 = a primary amine + analdehyde + H2O2",
"targ_desciption": "Catalyzes the oxidative deamination of biogenic andxenobiotic amines and has important functions in the metabolism ofneuroactive and vasoactive amines in the central nervous system andperipheral tissues. MAOB preferentially degrades benzylamine andphenylethylamine.",
"references": []
},
{
"targ_id": "T460",
"parent_targ_id": "T",
"full_name": "Amine oxidase [flavin-containing] A",
"abbrev": "MAO-A",
"protein_names": "Amine oxidase [flavin-containing] A (EC 1.4.3.4) (Monoamine oxidase type A) (MAO-A)",
"related_func_ids": "",
"category": "enzyme",
"subcategories": "Oxidoreductase",
"Uniport_ID": "P21397",
"Uniprot_name": "AOFA_HUMAN",
"EC_numbers": "1.4.3.4",
"gene_symbol": "MAOA",
"gene_synonyms": "",
"gene_synonyms_links": "",
"gene_name": "Monoamine oxidase A",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000189221",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion outer membrane; Single-pass type IV membrane protein; Cytoplasmic side",
"reaction": "a secondary aliphatic amine + H2O + O2 = a primary amine + analdehyde + H2O2",
"targ_desciption": "Catalyzes the oxidative deamination of biogenic andxenobiotic amines and has important functions in the metabolism ofneuroactive and vasoactive amines in the central nervous system andperipheral tissues. MAOA preferentially oxidizes biogenic amines suchas 5-hydroxytryptamine (5-HT), norepinephrine and epinephrine.",
"references": []
},
{
"targ_id": "T461",
"parent_targ_id": "T",
"full_name": "Aldehyde dehydrogenase, mitochondrial",
"abbrev": "ALDHI",
"protein_names": "Aldehyde dehydrogenase, mitochondrial (EC 1.2.1.3) (ALDH class 2) (ALDH-E2) (ALDHI)",
"related_func_ids": "",
"category": "enzyme",
"subcategories": "Oxidoreductase",
"Uniport_ID": "P05091",
"Uniprot_name": "ALDH2_HUMAN",
"EC_numbers": "1.2.1.3",
"gene_symbol": "ALDH2",
"gene_synonyms": "",
"gene_synonyms_links": "",
"gene_name": "Aldehyde dehydrogenase 2 family (mitochondrial)",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000111275",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion matrix",
"reaction": "an aldehyde + H2O + NAD(+) = a carboxylate + 2 H(+) + NADH",
"targ_desciption": "",
"references": []
},
{
"targ_id": "T462",
"parent_targ_id": "T",
"full_name": "5-aminolevulinate synthase, erythroid-specific, mitochondrial",
"abbrev": "ALAS-E",
"protein_names": "5-aminolevulinate synthase, erythroid-specific, mitochondrial (ALAS-E) (EC 2.3.1.37) (5-aminolevulinic acid synthase 2) (Delta-ALA synthase 2) (Delta-aminolevulinate synthase 2)",
"related_func_ids": "F021205",
"category": "enzyme",
"subcategories": "Acyltransferase, Transferase",
"Uniport_ID": "P22557",
"Uniprot_name": "HEM0_HUMAN",
"EC_numbers": "2.3.1.37",
"gene_symbol": "ALAS2",
"gene_synonyms": "ASB",
"gene_synonyms_links": "",
"gene_name": "5'-aminolevulinate synthase 2",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000158578",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion matrix",
"reaction": "glycine + H(+) + succinyl-CoA = 5-aminolevulinate + CO2 + CoA",
"targ_desciption": "",
"references": []
},
{
"targ_id": "T463",
"parent_targ_id": "T",
"full_name": "Sideroflexin-4",
"abbrev": "Breast cancer resistance marker 1",
"protein_names": "Sideroflexin-4 (Breast cancer resistance marker 1)",
"related_func_ids": "F021205",
"category": "protein",
"subcategories": "Transporter",
"Uniport_ID": "Q6P4A7",
"Uniprot_name": "SFXN4_HUMAN",
"EC_numbers": "",
"gene_symbol": "SFXN4",
"gene_synonyms": "SLC56A4",
"gene_synonyms_links": "",
"gene_name": "Sideroflexin 4",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000183605",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Multi-pass membrane protein",
"reaction": "",
"targ_desciption": "Mitochondrial amino-acid transporter (By similarity). Doesnot act as a serine transporter: not able to mediate transport ofserine into mitochondria (PubMed:30442778).{ECO:0000250|UniProtKB:Q9H9B4, ECO:0000269|PubMed:30442778}.",
"references": []
},
{
"targ_id": "T464",
"parent_targ_id": "T",
"full_name": "Mitochondrial glycine transporter",
"abbrev": "Solute carrier family 25 member 38",
"protein_names": "Mitochondrial glycine transporter (Mitochondrial glycine transporter GlyC) (Solute carrier family 25 member 38)",
"related_func_ids": "F021205",
"category": "protein",
"subcategories": "Transporter",
"Uniport_ID": "Q96DW6",
"Uniprot_name": "S2538_HUMAN",
"EC_numbers": "",
"gene_symbol": "SLC25A38",
"gene_synonyms": "FLJ20551",
"gene_synonyms_links": "",
"gene_name": "Solute carrier family 25 member 38",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000144659",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Multi-pass membrane protein",
"reaction": "",
"targ_desciption": "Mitochondrial glycine transporter that imports glycine intothe mitochondrial matrix. Plays an important role in providing glycinefor the first enzymatic step in heme biosynthesis, the condensation ofglycine with succinyl-CoA to produce 5-aminolevulinate (ALA) in themitochondrial matrix. Required during erythropoiesis.{ECO:0000255|HAMAP-Rule:MF_03064, ECO:0000269|PubMed:19412178,ECO:0000269|PubMed:27476175}.",
"references": []
},
{
"targ_id": "T465",
"parent_targ_id": "T",
"full_name": "5-aminolevulinate synthase, nonspecific, mitochondrial",
"abbrev": "ALAS-H",
"protein_names": "5-aminolevulinate synthase, nonspecific, mitochondrial (ALAS-H) (EC 2.3.1.37) (5-aminolevulinic acid synthase 1) (Delta-ALA synthase 1) (Delta-aminolevulinate synthase 1)",
"related_func_ids": "F021205",
"category": "enzyme",
"subcategories": "Acyltransferase, Transferase",
"Uniport_ID": "P13196",
"Uniprot_name": "HEM1_HUMAN",
"EC_numbers": "2.3.1.37",
"gene_symbol": "ALAS1",
"gene_synonyms": "ALAS, ALAS3",
"gene_synonyms_links": "",
"gene_name": "5'-aminolevulinate synthase 1",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000023330",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion matrix",
"reaction": "glycine + H(+) + succinyl-CoA = 5-aminolevulinate + CO2 + CoA",
"targ_desciption": "",
"references": []
},
{
"targ_id": "T466",
"parent_targ_id": "T",
"full_name": "5-aminolevulinate synthase",
"abbrev": "5-aminolevulinic acid synthase",
"protein_names": "5-aminolevulinate synthase (EC 2.3.1.37) (5-aminolevulinic acid synthase) (Delta-ALA synthase) (Delta-aminolevulinate synthase)",
"related_func_ids": "F021205",
"category": "enzyme",
"subcategories": "",
"Uniport_ID": "Q5JAM2",
"Uniprot_name": "Q5JAM2_HUMAN",
"EC_numbers": "2.3.1.37",
"gene_symbol": "ALAS1 hCG_42575",
"gene_synonyms": "",
"gene_synonyms_links": "",
"gene_name": "",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion matrix",
"reaction": "glycine + H(+) + succinyl-CoA = 5-aminolevulinate + CO2 + CoA",
"targ_desciption": "",
"references": []
},
{
"targ_id": "T467",
"parent_targ_id": "T",
"full_name": "Ferrochelatase, mitochondrial",
"abbrev": "Heme synthase",
"protein_names": "Ferrochelatase, mitochondrial (EC 4.99.1.1) (Heme synthase) (Protoheme ferro-lyase)",
"related_func_ids": "F021205",
"category": "enzyme",
"subcategories": "Lyase",
"Uniport_ID": "P22830",
"Uniprot_name": "HEMH_HUMAN",
"EC_numbers": "4.99.1.1",
"gene_symbol": "FECH",
"gene_synonyms": "",
"gene_synonyms_links": "",
"gene_name": "Ferrochelatase",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000066926",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Peripheral membrane protein; Matrix side",
"reaction": "2 H(+) + heme b = Fe(2+) + protoporphyrin IX",
"targ_desciption": "Catalyzes the ferrous insertion into protoporphyrin IX.{ECO:0000269|PubMed:27599036, ECO:0000269|PubMed:8276824}.",
"references": []
},
{
"targ_id": "T468",
"parent_targ_id": "T",
"full_name": "Aldehyde dehydrogenase X, mitochondrial",
"abbrev": "Aldehyde dehydrogenase 5",
"protein_names": "Aldehyde dehydrogenase X, mitochondrial (EC 1.2.1.3) (Aldehyde dehydrogenase 5) (Aldehyde dehydrogenase family 1 member B1)",
"related_func_ids": "F020603",
"category": "enzyme",
"subcategories": "Oxidoreductase",
"Uniport_ID": "P30837",
"Uniprot_name": "AL1B1_HUMAN",
"EC_numbers": "1.2.1.3",
"gene_symbol": "ALDH1B1",
"gene_synonyms": "ALDH5, ALDHX",
"gene_synonyms_links": "",
"gene_name": "Aldehyde dehydrogenase 1 family member B1",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000137124",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion matrix",
"reaction": "an aldehyde + H2O + NAD(+) = a carboxylate + 2 H(+) + NADH",
"targ_desciption": "ALDHs play a major role in the detoxification of alcohol-derived acetaldehyde. They are involved in the metabolism ofcorticosteroids, biogenic amines, neurotransmitters, and lipidperoxidation.",
"references": []
},
{
"targ_id": "T469",
"parent_targ_id": "T",
"full_name": "Delta-1-pyrroline-5-carboxylate synthase",
"abbrev": "P5CS",
"protein_names": "Delta-1-pyrroline-5-carboxylate synthase (P5CS) (Aldehyde dehydrogenase family 18 member A1) [Includes: Glutamate 5-kinase (GK) (EC 2.7.2.11) (Gamma-glutamyl kinase); Gamma-glutamyl phosphate reductase (GPR) (EC 1.2.1.41) (Glutamate-5-semialdehyde dehydrogenase) (Glutamyl-gamma-semialdehyde dehydrogenase)]",
"related_func_ids": "F020707; F020715",
"category": "enzyme",
"subcategories": "Kinase, Multifunctional enzyme, Oxidoreductase, Transferase",
"Uniport_ID": "P54886",
"Uniprot_name": "P5CS_HUMAN",
"EC_numbers": "2.7.2.11; 1.2.1.41",
"gene_symbol": "ALDH18A1",
"gene_synonyms": "GSAS, P5CS, PYCS, SPG9",
"gene_synonyms_links": "",
"gene_name": "Aldehyde dehydrogenase 18 family member A1",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000059573",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane",
"reaction": "ATP + L-glutamate = ADP + L-glutamyl 5-phosphate; L-glutamate 5-semialdehyde + NADP(+) + phosphate = H(+) + L-glutamyl 5-phosphate + NADPH",
"targ_desciption": "Bifunctional enzyme that converts glutamate to glutamate 5-semialdehyde, an intermediate in the biosynthesis of proline, ornithineand arginine. {ECO:0000269|PubMed:10037775,ECO:0000269|PubMed:11092761, ECO:0000269|PubMed:26297558,ECO:0000269|PubMed:26320891}.",
"references": []
},
{
"targ_id": "T470",
"parent_targ_id": "T",
"full_name": "D-beta-hydroxybutyrate dehydrogenase, mitochondrial",
"abbrev": "BDH",
"protein_names": "D-beta-hydroxybutyrate dehydrogenase, mitochondrial (EC 1.1.1.30) (3-hydroxybutyrate dehydrogenase) (BDH) (Short chain dehydrogenase/reductase family 9C member 1)",
"related_func_ids": "F02060201",
"category": "enzyme",
"subcategories": "Allosteric enzyme, Oxidoreductase",
"Uniport_ID": "Q02338",
"Uniprot_name": "BDH_HUMAN",
"EC_numbers": "1.1.1.30",
"gene_symbol": "BDH1",
"gene_synonyms": "BDH, SDR9C1",
"gene_synonyms_links": "",
"gene_name": "3-hydroxybutyrate dehydrogenase 1",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000161267",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion inner membrane; Mitochondrion matrix",
"reaction": "(R)-3-hydroxybutanoate + NAD(+) = acetoacetate + H(+) + NADH",
"targ_desciption": "",
"references": []
},
{
"targ_id": "T471",
"parent_targ_id": "T",
"full_name": "Carbonic anhydrase 5A, mitochondrial",
"abbrev": "CA-VA",
"protein_names": "Carbonic anhydrase 5A, mitochondrial (EC 4.2.1.1) (Carbonate dehydratase VA) (Carbonic anhydrase VA) (CA-VA)",
"related_func_ids": "F02110312",
"category": "enzyme",
"subcategories": "Lyase",
"Uniport_ID": "P35218",
"Uniprot_name": "CAH5A_HUMAN",
"EC_numbers": "4.2.1.1",
"gene_symbol": "CA5A",
"gene_synonyms": "CA5, CAV, CAVA",
"gene_synonyms_links": "",
"gene_name": "Carbonic anhydrase 5A",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000174990",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion",
"reaction": "H(+) + hydrogencarbonate = CO2 + H2O",
"targ_desciption": "Reversible hydration of carbon dioxide. Low activity.",
"references": []
},
{
"targ_id": "T472",
"parent_targ_id": "T",
"full_name": "C-terminal-binding protein 1",
"abbrev": "CtBP1",
"protein_names": "C-terminal-binding protein 1 (CtBP1) (EC 1.1.1.-)",
"related_func_ids": "F060301",
"category": "protein",
"subcategories": "",
"Uniport_ID": "Q13363",
"Uniprot_name": "CTBP1_HUMAN",
"EC_numbers": "1.1.1.-",
"gene_symbol": "CTBP1 CTBP",
"gene_synonyms": "",
"gene_synonyms_links": "",
"gene_name": "",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "",
"omim_id": "",
"genecard_link": "",
"locs": "Cytoplasm; Nucleus",
"reaction": "",
"targ_desciption": "Corepressor targeting diverse transcription regulators suchas GLIS2 or BCL6. Has dehydrogenase activity. Involved in controllingthe equilibrium between tubular and stacked structures in the Golgicomplex. Functions in brown adipose tissue (BAT) differentiation.{ECO:0000269|PubMed:12419229, ECO:0000269|PubMed:15542832,ECO:0000269|PubMed:18212045, ECO:0000269|PubMed:19103759,ECO:0000269|PubMed:9858600}.",
"references": []
},
{
"targ_id": "T473",
"parent_targ_id": "T",
"full_name": "Complement component 1 Q subcomponent-binding protein, mitochondrial",
"abbrev": "SF2AP32",
"protein_names": "Complement component 1 Q subcomponent-binding protein, mitochondrial (ASF/SF2-associated protein p32) (Glycoprotein gC1qBP) (C1qBP) (Hyaluronan-binding protein 1) (Mitochondrial matrix protein p32) (gC1q-R protein) (p33) (SF2AP32)",
"related_func_ids": "F060301; F060302",
"category": "protein",
"subcategories": "",
"Uniport_ID": "Q07021",
"Uniprot_name": "C1QBP_HUMAN",
"EC_numbers": "",
"gene_symbol": "C1QBP",
"gene_synonyms": "gC1Q-R, gC1qR, HABP1, p32, SF2p32",
"gene_synonyms_links": "",
"gene_name": "Complement C1q binding protein",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000108561",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion matrix; Nucleus; Cell membrane; Peripheral membrane protein; Extracellular side; Secreted; Cytoplasm; Nucleus, nucleolus",
"reaction": "",
"targ_desciption": "Is believed to be a multifunctional and multicompartmentalprotein involved in inflammation and infection processes, ribosomebiogenesis, protein synthesis in mitochondria, regulation of apoptosis,transcriptional regulation and pre-mRNA splicing. At the cell surfaceis thought to act as an endothelial receptor for plasma proteins of thecomplement and kallikrein-kinin cascades. Putative receptor for C1q.specifically binds to the globular 'heads' of C1q thus inhibiting C1.may perform the receptor function through a complex with C1qR/CD93. Incomplex with cytokeratin-1/KRT1 is a high affinity receptor forkininogen-1/HMWK. Can also bind other plasma proteins, such ascoagulation factor XII leading to its autoactivation. May function tobind initially fluid kininogen-1 to the cell membrane. The secretedform may enhance both extrinsic and intrinsic coagulation pathways. Itis postulated that the cell surface form requires docking withtransmembrane proteins for downstream signaling which might be specificfor a cell-type or response. By acting as C1q receptor is involved inchemotaxis of immature dendritic cells and neutrophils and is proposedto signal through CD209/DC-SIGN on immature dendritic cells, throughintegrin alpha-4/beta-1 during trophoblast invasion of the decidua, andthrough integrin beta-1 during endothelial cell adhesion and spreading.Signaling involved in inhibition of innate immune response isimplicating the PI3K-AKT/PKB pathway. Required for protein synthesis inmitochondria (PubMed:28942965). In mitochondrial translation may beinvolved in formation of functional 55S mitoribosomes. the functionseems to involve its RNA-binding activity. May be involved in thenucleolar ribosome maturation process. the function may involve theexchange of FBL for RRP1 in the association with pre-ribosomeparticles. Involved in regulation of RNA splicing by inhibiting theRNA-binding capacity of SRSF1 and its phosphorylation. Is required forthe nuclear translocation of splicing factor U2AF1L4. Involved inregulation of CDKN2A- and HRK-mediated apoptosis. Stabilizesmitochondrial CDKN2A isoform smARF. May be involved in regulation ofFOXC1 transcriptional activity and NFY/CCAAT-binding factor complex-mediated transcription. May play a role in antibacterial defense as itcan bind to cell surface hyaluronan and inhibit Streptococcuspneumoniae hyaluronate lyase. May be involved in modulation of theimmune response. ligation by HCV core protein is resulting insuppression of interleukin-12 production in monocyte-derived dendriticcells. Involved in regulation of antiviral response by inhibitingDDX58- and IFIH1-mediated signaling pathways probably involving itsassociation with MAVS after viral infection.{ECO:0000269|PubMed:10022843, ECO:0000269|PubMed:10479529,ECO:0000269|PubMed:10722602, ECO:0000269|PubMed:11086025,ECO:0000269|PubMed:11859136, ECO:0000269|PubMed:15243141,ECO:0000269|PubMed:16140380, ECO:0000269|PubMed:16177118,ECO:0000269|PubMed:17881511, ECO:0000269|PubMed:18676636,ECO:0000269|PubMed:19004836, ECO:0000269|PubMed:19164550,ECO:0000269|PubMed:20810993, ECO:0000269|PubMed:21536856,ECO:0000269|PubMed:21544310, ECO:0000269|PubMed:22700724,ECO:0000269|PubMed:28942965, ECO:0000269|PubMed:8662673,ECO:0000269|PubMed:8710908, ECO:0000269|PubMed:9461517}.; (Microbial infection) Involved in HIV-1 replication,presumably by contributing to splicing of viral RNA.{ECO:0000269|PubMed:12833064}.; (Microbial infection) In infection processes acts as anattachment site for microbial proteins, including Listeriamonocytogenes internalin B (InlB) and Staphylococcus aureus protein A.{ECO:0000269|PubMed:10722602, ECO:0000269|PubMed:10747014,ECO:0000269|PubMed:12411480}.; (Microbial infection) Involved in replication of Rubellavirus. {ECO:0000269|PubMed:12034482}.",
"references": []
},
{
"targ_id": "T474",
"parent_targ_id": "T",
"full_name": "Protein C19orf12",
"abbrev": "",
"protein_names": "Protein C19orf12",
"related_func_ids": "F0701; F0703",
"category": "protein",
"subcategories": "",
"Uniport_ID": "Q9NSK7",
"Uniprot_name": "CS012_HUMAN",
"EC_numbers": "",
"gene_symbol": "C19orf12",
"gene_synonyms": "DKFZP762D096, MGC10922, MPAN, NBIA4, SPG43",
"gene_synonyms_links": "",
"gene_name": "Chromosome 19 open reading frame 12",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000131943",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion; Mitochondrion membrane; Single-pass membrane protein; Endoplasmic reticulum; Cytoplasm, cytosol",
"reaction": "",
"targ_desciption": "",
"references": []
},
{
"targ_id": "T475",
"parent_targ_id": "T",
"full_name": "Netrin receptor DCC",
"abbrev": "Colorectal cancer suppressor",
"protein_names": "Netrin receptor DCC (Colorectal cancer suppressor) (Immunoglobulin superfamily DCC subclass member 1) (Tumor suppressor protein DCC)",
"related_func_ids": "F0701",
"category": "enzyme",
"subcategories": "",
"Uniport_ID": "P43146",
"Uniprot_name": "DCC_HUMAN",
"EC_numbers": "",
"gene_symbol": "DCC IGDCC1",
"gene_synonyms": "",
"gene_synonyms_links": "",
"gene_name": "",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "",
"omim_id": "",
"genecard_link": "",
"locs": "Membrane; Single-pass type I membrane protein",
"reaction": "",
"targ_desciption": "Receptor for netrin required for axon guidance. Mediates axonattraction of neuronal growth cones in the developing nervous systemupon ligand binding. Its association with UNC5 proteins may triggersignaling for axon repulsion. It also acts as a dependence receptorrequired for apoptosis induction when not associated with netrinligand. Implicated as a tumor suppressor gene.{ECO:0000269|PubMed:8187090, ECO:0000269|PubMed:8861902}.",
"references": []
},
{
"targ_id": "T476",
"parent_targ_id": "T",
"full_name": "Protein diaphanous homolog 1",
"abbrev": "DRF1",
"protein_names": "Protein diaphanous homolog 1 (Diaphanous-related formin-1) (DRF1)",
"related_func_ids": "F0807",
"category": "protein",
"subcategories": "",
"Uniport_ID": "O60610",
"Uniprot_name": "DIAP1_HUMAN",
"EC_numbers": "",
"gene_symbol": "DIAPH1 DIAP1",
"gene_synonyms": "",
"gene_synonyms_links": "",
"gene_name": "",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "",
"omim_id": "",
"genecard_link": "",
"locs": "Cell membrane; Cell projection, ruffle membrane; Cytoplasm, cytoskeleton; Cytoplasm, cytoskeleton, microtubule organizing center, centrosome; Cytoplasm, cytoskeleton, spindle; Cytoplasm; Nucleus",
"reaction": "",
"targ_desciption": "Actin nucleation and elongation factor required for theassembly of F-actin structures, such as actin cables and stress fibers(By similarity). Binds to the barbed end of the actin filament andslows down actin polymerization and depolymerization (By similarity).Required for cytokinesis, and transcriptional activation of the serumresponse factor (By similarity). DFR proteins couple Rho and Srctyrosine kinase during signaling and the regulation of actin dynamics(By similarity). Functions as a scaffold protein for MAPRE1 and APC tostabilize microtubules and promote cell migration (By similarity). Hasneurite outgrowth promoting activity. Acts in a Rho-dependent manner torecruit PFY1 to the membrane (By similarity). In hear cells, it mayplay a role in the regulation of actin polymerization in hair cells(PubMed:20937854, PubMed:21834987, PubMed:26912466). The MEMO1-RHOA-DIAPH1 signaling pathway plays an important role in ERBB2-dependentstabilization of microtubules at the cell cortex (PubMed:20937854,PubMed:21834987). It controls the localization of APC and CLASP2 to thecell membrane, via the regulation of GSK3B activity (PubMed:20937854,PubMed:21834987). In turn, membrane-bound APC allows the localizationof the MACF1 to the cell membrane, which is required for microtubulecapture and stabilization (PubMed:20937854, PubMed:21834987). Plays arole in the regulation of cell morphology and cytoskeletalorganization. Required in the control of cell shape (PubMed:20937854,PubMed:21834987). Plays a role in brain development (PubMed:24781755).Also acts as an actin nucleation and elongation factor in the nucleusby promoting nuclear actin polymerization inside the nucleus to driveserum-dependent SRF-MRTFA activity (By similarity).{ECO:0000250|UniProtKB:O08808, ECO:0000269|PubMed:20937854,ECO:0000269|PubMed:21834987, ECO:0000269|PubMed:24781755,ECO:0000269|PubMed:26912466}.",
"references": []
},
{
"targ_id": "T479",
"parent_targ_id": "T",
"full_name": "Optic atrophy 3 protein",
"abbrev": "",
"protein_names": "Optic atrophy 3 protein",
"related_func_ids": "F0206",
"category": "protein",
"subcategories": "",
"Uniport_ID": "Q9H6K4",
"Uniprot_name": "OPA3_HUMAN",
"EC_numbers": "",
"gene_symbol": "OPA3",
"gene_synonyms": "FLJ22187, MGA3",
"gene_synonyms_links": "",
"gene_name": "OPA3, outer mitochondrial membrane lipid metabolism regulator",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000125741",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion",
"reaction": "",
"targ_desciption": "May play some role in mitochondrial processes.",
"references": []
},
{
"targ_id": "T480",
"parent_targ_id": "T",
"full_name": "Patatin-like phospholipase domain-containing protein 4",
"abbrev": "Protein GS2",
"protein_names": "Patatin-like phospholipase domain-containing protein 4 (EC 3.1.1.3) (Protein GS2)",
"related_func_ids": "F0206",
"category": "protein",
"subcategories": "",
"Uniport_ID": "P41247",
"Uniprot_name": "PLPL4_HUMAN",
"EC_numbers": "3.1.1.3",
"gene_symbol": "PNPLA4 DXS1283E GS2",
"gene_synonyms": "",
"gene_synonyms_links": "",
"gene_name": "",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion",
"reaction": "a triacylglycerol + H2O = a diacylglycerol + a fatty acid +H(+); 2 1-(9Z-octadecenoyl)-glycerol = 1,2-di-(9Z-octadecenoyl)-glycerol + glycerol; 1,2-di-(9Z-octadecenoyl)-glycerol + 1-(9Z-octadecenoyl)-glycerol = 1,2,3-tri-(9Z-octadecenoyl)-glycerol + glycerol; 1,3-di-(9Z-octadecenoyl)-glycerol + 1-(9Z-octadecenoyl)-glycerol = 1,2,3-tri-(9Z-octadecenoyl)-glycerol + glycerol; (9Z)-octadecenoate + 1,2-di-(9Z-octadecenoyl)-glycerol + H(+)= 1,2,3-tri-(9Z-octadecenoyl)-glycerol + H2O; 1,2,3-tri-(9Z-octadecenoyl)-glycerol + H2O = (9Z)-octadecenoate + 1,3-di-(9Z-octadecenoyl)-glycerol + H(+); all-trans-retinyl hexadecanoate + H2O = all-trans-retinol +H(+) + hexadecanoate; 1,2,3-tri-(9Z-octadecenoyl)-glycerol + all-trans-retinol =all-trans-retinyl 9Z-octadecenoate + di-(9Z)-octadecenoylglycerol",
"targ_desciption": "Has abundant triacylglycerol lipase activity(PubMed:15364929). Transfers fatty acid from triglyceride to retinol,hydrolyzes retinylesters, and generates 1,3-diacylglycerol fromtriglycerides (PubMed:17603008). {ECO:0000269|PubMed:15364929,ECO:0000269|PubMed:17603008}.",
"references": []
},
{
"targ_id": "T481",
"parent_targ_id": "T",
"full_name": "Calcium-independent phospholipase A2-gamma",
"abbrev": "iPLA2-gamma",
"protein_names": "Calcium-independent phospholipase A2-gamma (EC 3.1.1.5) (Intracellular membrane-associated calcium-independent phospholipase A2 gamma) (iPLA2-gamma) (PNPLA-gamma) (Patatin-like phospholipase domain-containing protein 8) (iPLA2-2)",
"related_func_ids": "F020601; F07",
"category": "enzyme",
"subcategories": "Hydrolase",
"Uniport_ID": "Q9NP80",
"Uniprot_name": "PLPL8_HUMAN",
"EC_numbers": "3.1.1.5",
"gene_symbol": "PNPLA8",
"gene_synonyms": "IPLA2-2, IPLA2G, iPLA2gamma",
"gene_synonyms_links": "",
"gene_name": "Patatin like phospholipase domain containing 8",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000135241",
"omim_id": "",
"genecard_link": "",
"locs": "Endoplasmic reticulum membrane; Single-pass membrane protein; Microsome membrane; Single-pass membrane protein; Mitochondrion membrane; Single-pass membrane protein; Peroxisome membrane; Single-pass membrane protein",
"reaction": "a 1-acyl-sn-glycero-3-phosphocholine + H2O = a fatty acid +H(+) + sn-glycerol 3-phosphocholine; 1-acyl-2-(9Z,12Z)-octadecadienoyl-sn-glycero-3-phosphoethanolamine + H2O = (9Z,12Z)-octadecadienoate + a 1-acyl-sn-glycero-3-phosphoethanolamine + H(+); 1-acyl-2-(9Z,12Z)-octadecadienoyl-sn-glycero-3-phosphocholine+ H2O = (9Z,12Z)-octadecadienoate + a 1-acyl-sn-glycero-3-phosphocholine + H(+); 1-acyl-2-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-sn-glycero-3-phosphoethanolamine + H2O = (5Z,8Z,11Z,14Z)-eicosatetraenoate + a 1-acyl-sn-glycero-3-phosphoethanolamine + H(+); 1-acyl-2-(5Z,8Z,11Z,14Z-eicosatetraenoyl)-sn-glycero-3-phosphocholine + H2O = (5Z,8Z,11Z,14Z)-eicosatetraenoate + a 1-acyl-sn-glycero-3-phosphocholine + H(+); 1-hexadecanoyl-2-(5Z,8Z,11Z,14Z-eicosatetraenoyl)-sn-glycero-3-phosphocholine + H2O = 2-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-sn-glycero-3-phosphocholine + H(+) + hexadecanoate; 1-O-(1Z)-hexadecenyl-2 (5Z,8Z,11Z,14Z)-eicosatetraenoyl-sn-glycero-3-phosphocholine + H2O = (5Z,8Z,11Z,14Z)-eicosatetraenoate +1-(1Z-hexadecenyl)-sn-glycero-3-phosphocholine + H(+); 1-hexadecanoyl-sn-glycero-3-phosphocholine + H2O = H(+) +hexadecanoate + sn-glycerol 3-phosphocholine; 1-octadecanoyl-2-(9Z-octadecenoyl)-sn-glycero-3-phosphocholine+ H2O = (9Z)-octadecenoate + 1-octadecanoyl-sn-glycero-3-phosphocholine + H(+); 1-octadecanoyl-2-(9Z-octadecenoyl)-sn-glycero-3-phosphocholine+ H2O = 2-(9Z-octadecenoyl)-sn-glycero-3-phosphocholine + H(+) +octadecanoate; 1-hexadecanoyl-2-(4Z,7Z,10Z,13Z,16Z,19Z-docosahexaenoyl)-sn-glycero-3-phosphocholine + H2O = 2-(4Z,7Z,10Z,13Z,16Z,19Z-docosahexaenoyl)-sn-glycero-3-phosphocholine + H(+) + hexadecanoate; 1-hexadecanoyl-2-(9Z-octadecenoyl)-sn-glycero-3-phosphocholine+ H2O = (9Z)-octadecenoate + 1-hexadecanoyl-sn-glycero-3-phosphocholine + H(+); 1-hexadecanoyl-2-(5Z,8Z,11Z,14Z-eicosatetraenoyl)-sn-glycero-3-phosphoethanolamine + H2O = (5Z,8Z,11Z,14Z)-eicosatetraenoate + 1-hexadecanoyl-sn-glycero-3-phosphoethanolamine + H(+)",
"targ_desciption": "Calcium-independent phospholipase A2, which promotes cellularmembrane hydrolysis and prostaglandin production (PubMed:10744668,PubMed:15695510). Catalyzes the hydrolysis of the sn-2 position ofglycerophospholipids, phosphytidylserine and to a lower extentphosphatidylcholine (PubMed:10744668). Cleaves membrane phospholipids(PubMed:15695510). Participates in the generation of lipid secondmessengers through the mobilization of arachidonic acid in response tocellular stimuli. Synthesizes 2-arachidonoyl lysophosphatidylcholine, akey branch point metabolite in eicosanoid signaling (PubMed:15908428).Participates in the lipid plasticity of myocardium, plays a role in thegeneration of signaling metabolites and has a prominent effect on themodulaton of energy storage and utilization (PubMed:17213206).Essential for maintaining efficient bioenergetic mitochondrial functionthrough tailoring mitochondrial membrane lipid metabolism andcomposition (By similarity). {ECO:0000250|UniProtKB:Q8K1N1,ECO:0000269|PubMed:10744668, ECO:0000269|PubMed:15695510,ECO:0000269|PubMed:15908428, ECO:0000269|PubMed:17213206}.",
"references": []
},
{
"targ_id": "T483",
"parent_targ_id": "T",
"full_name": "Inorganic pyrophosphatase 2, mitochondrial",
"abbrev": "PPase 2",
"protein_names": "Inorganic pyrophosphatase 2, mitochondrial (EC 3.6.1.1) (Pyrophosphatase SID6-306) (Pyrophosphate phospho-hydrolase 2) (PPase 2)",
"related_func_ids": "F060303; F01",
"category": "enzyme",
"subcategories": "Hydrolase",
"Uniport_ID": "Q9H2U2",
"Uniprot_name": "IPYR2_HUMAN",
"EC_numbers": "3.6.1.1",
"gene_symbol": "PPA2",
"gene_synonyms": "FLJ20459",
"gene_synonyms_links": "",
"gene_name": "Pyrophosphatase (inorganic) 2",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000138777",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion",
"reaction": "diphosphate + H2O = H(+) + 2 phosphate",
"targ_desciption": "Hydrolyzes inorganic pyrophosphate (PubMed:27523597). Thisactivity is essential for correct regulation of mitochondrial membranepotential, and mitochondrial organization and function(PubMed:27523598). {ECO:0000269|PubMed:27523597,ECO:0000269|PubMed:27523598}.",
"references": []
},
{
"targ_id": "T484",
"parent_targ_id": "T",
"full_name": "Major prion protein",
"abbrev": "PrP",
"protein_names": "Major prion protein (PrP) (ASCR) (PrP27-30) (PrP33-35C) (CD antigen CD230)",
"related_func_ids": "F02110301; F02110304",
"category": "protein",
"subcategories": "",
"Uniport_ID": "P04156",
"Uniprot_name": "PRIO_HUMAN",
"EC_numbers": "",
"gene_symbol": "PRNP ALTPRP PRIP PRP",
"gene_synonyms": "",
"gene_synonyms_links": "",
"gene_name": "",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "",
"omim_id": "",
"genecard_link": "",
"locs": "Cell membrane; Lipid-anchor, GPI-anchor; Golgi apparatus",
"reaction": "",
"targ_desciption": "Its primary physiological function is unclear. May play arole in neuronal development and synaptic plasticity. May be requiredfor neuronal myelin sheath maintenance. May promote myelin homeostasisthrough acting as an agonist for ADGRG6 receptor. May play a role iniron uptake and iron homeostasis. Soluble oligomers are toxic tocultured neuroblastoma cells and induce apoptosis (in vitro) (Bysimilarity). Association with GPC1 (via its heparan sulfate chains)targets PRNP to lipid rafts. Also provides Cu(2+) or ZN(2+) for theascorbate-mediated GPC1 deaminase degradation of its heparan sulfateside chains (By similarity). {ECO:0000250|UniProtKB:P04925,ECO:0000269|PubMed:12732622, ECO:0000269|PubMed:19936054,ECO:0000269|PubMed:20564047, ECO:0000305}.",
"references": []
},
{
"targ_id": "T486",
"parent_targ_id": "T",
"full_name": "Sulfite oxidase, mitochondrial",
"abbrev": "",
"protein_names": "Sulfite oxidase, mitochondrial (EC 1.8.3.1)",
"related_func_ids": "F0222",
"category": "enzyme",
"subcategories": "Oxidoreductase",
"Uniport_ID": "P51687",
"Uniprot_name": "SUOX_HUMAN",
"EC_numbers": "1.8.3.1",
"gene_symbol": "SUOX",
"gene_synonyms": "",
"gene_synonyms_links": "",
"gene_name": "Sulfite oxidase",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000139531",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion intermembrane space",
"reaction": "H2O + O2 + sulfite = H2O2 + sulfate",
"targ_desciption": "",
"references": []
},
{
"targ_id": "T487",
"parent_targ_id": "T",
"full_name": "Carbonic anhydrase 5B, mitochondrial",
"abbrev": "CA-VB",
"protein_names": "Carbonic anhydrase 5B, mitochondrial (EC 4.2.1.1) (Carbonate dehydratase VB) (Carbonic anhydrase VB) (CA-VB)",
"related_func_ids": "F021103",
"category": "enzyme",
"subcategories": "Lyase",
"Uniport_ID": "Q9Y2D0",
"Uniprot_name": "CAH5B_HUMAN",
"EC_numbers": "4.2.1.1",
"gene_symbol": "CA5B",
"gene_synonyms": "",
"gene_synonyms_links": "",
"gene_name": "Carbonic anhydrase 5B",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "ENSG00000169239",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion",
"reaction": "H(+) + hydrogencarbonate = CO2 + H2O",
"targ_desciption": "Reversible hydration of carbon dioxide.",
"references": []
},
{
"targ_id": "T488",
"parent_targ_id": "T",
"full_name": "Endonuclease G, mitochondrial",
"abbrev": "",
"protein_names": "Endonuclease G, mitochondrial",
"related_func_ids": "F0701",
"category": "enzyme",
"subcategories": "Endonuclease, Hydrolase, Nuclease",
"Uniport_ID": " Q14249 ",
"Uniprot_name": "NUCG_HUMAN",
"EC_numbers": "",
"gene_symbol": "ENDOG",
"gene_synonyms": "",
"gene_synonyms_links": "",
"gene_name": "",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "",
"omim_id": "",
"genecard_link": "",
"locs": "Mitochondrion",
"reaction": "",
"targ_desciption": "Cleaves DNA at double-stranded (DG)n.(DC)n and at single-stranded (DC)n tracts. In addition to deoxyribonuclease activities, also has ribonuclease (RNase) and RNase H activities. ",
"references": []
},
{
"targ_id": "T489",
"parent_targ_id": "T",
"full_name": "Mitofusin-1",
"abbrev": "MFN1",
"protein_names": "Mitofusin-1",
"related_func_ids": "F030803",
"category": "enzyme",
"subcategories": "Hydrolase",
"Uniport_ID": "Q8IWA4",
"Uniprot_name": "MFN1_HUMAN",
"EC_numbers": "",
"gene_symbol": "MFN1",
"gene_synonyms": "",
"gene_synonyms_links": "",
"gene_name": "",
"hgnc_id": "",
"entrez_id": "",
"ensembl_id": "",
"omim_id": "",
"genecard_link": "",
"locs": "",
"reaction": "",
"targ_desciption": "",
"references": [
"RC03647",
"RC03783",
"RC03784",
"RC03785",
"RC03786",
"RC03787"
]
},
{
"targ_id": "T490",
"parent_targ_id": "T",
"full_name": "Mitochondrially Encoded TRNA-Lys (AAA/G)",
"abbrev": "MT-TK",
"protein_names": "",
"related_func_ids": "F060303",
"category": "RNA",
"subcategories": "tRNA",
"Uniport_ID": "",
"Uniprot_name": "",
"EC_numbers": "",
"gene_symbol": "MT-TK",
"gene_synonyms": "Mitochondrially Encoded TRNA-Lys (AAA/G); MT-TK; Mitochondrially Encoded TRNA Lysine; TRNA Lysine; TRNK; TRNA; MTTK; TrnK",
"gene_synonyms_links": "https://www.genenames.org/data/gene-symbol-report/#!/hgnc_id/7489||http://www.ensembl.org/Homo_sapiens/geneview?gene=ENSG00000210156; https://www.genenames.org/data/gene-symbol-report/#!/hgnc_id/7489||http://www.ensembl.org/Homo_sapiens/geneview?gene=ENSG00000210156; https://www.genenames.org/data/gene-symbol-report/#!/hgnc_id/7489; https://www.genenames.org/data/gene-symbol-report/#!/hgnc_id/7489; https://www.ncbi.nlm.nih.gov/gene/4566; https://www.ncbi.nlm.nih.gov/gene/4566; https://www.ncbi.nlm.nih.gov/gene/4566; https://www.genenames.org/data/gene-symbol-report/#!/hgnc_id/7489",
"gene_name": "",
"hgnc_id": "7489.0",
"entrez_id": "4566.0",
"ensembl_id": "ENSG00000210156",
"omim_id": "590060.0",
"genecard_link": "https://www.genecards.org/cgi-bin/carddisp.pl?gene=MT-TK",
"locs": "",
"reaction": "",
"targ_desciption": "",
"references": []
},
{
"targ_id": "T491",
"parent_targ_id": "T",
"full_name": "Mitochondrially Encoded TRNA-Leu (UUA/G) 1",
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