#ID(s) interactor A ID(s) interactor B Alt. ID(s) interactor A Alt. ID(s) interactor B Alias(es) interactor A Alias(es) interactor B Interaction detection method(s) Publication 1st author(s) Publication Identifier(s) Taxid interactor A Taxid interactor B Interaction type(s) Source database(s) Interaction identifier(s) Confidence value(s) Expansion method(s) Biological role(s) interactor A Biological role(s) interactor B Experimental role(s) interactor A Experimental role(s) interactor B Type(s) interactor A Type(s) interactor B Xref(s) interactor A Xref(s) interactor B Interaction Xref(s) Annotation(s) interactor A Annotation(s) interactor B Interaction annotation(s) Host organism(s) Interaction parameter(s) Creation date Update date Checksum(s) interactor A Checksum(s) interactor B Interaction Checksum(s) Negative Feature(s) interactor A Feature(s) interactor B Stoichiometry(s) interactor A Stoichiometry(s) interactor B Identification method participant A Identification method participant B uniprotkb:Q9HCC0 uniprotkb:Q96RQ3 intact:EBI-2211296|uniprotkb:A6NIY9|uniprotkb:Q96C27|uniprotkb:Q9Y4L7|ensembl:ENSP00000343657 intact:EBI-2211703|uniprotkb:Q59ES4|uniprotkb:Q9H959|uniprotkb:Q9NS97|ensembl:ENSP00000265594 psi-mi:mccb_human(display_long)|uniprotkb:MCCC2(gene name)|psi-mi:MCCC2(display_short)|uniprotkb:MCCB(gene name synonym)|uniprotkb:"3-methylcrotonyl-CoA:carbon dioxide ligase subunit beta"(gene name synonym)|uniprotkb:3-methylcrotonyl-CoA carboxylase non-biotin-containing subunit(gene name synonym)|uniprotkb:3-methylcrotonyl-CoA carboxylase 2(gene name synonym) psi-mi:mcca_human(display_long)|uniprotkb:MCCC1(gene name)|psi-mi:MCCC1(display_short)|uniprotkb:MCCA(gene name synonym)|uniprotkb:3-methylcrotonyl-CoA carboxylase 1(gene name synonym)|uniprotkb:"3-methylcrotonyl-CoA:carbon dioxide ligase subunit alpha"(gene name synonym)|uniprotkb:3-methylcrotonyl-CoA carboxylase biotin-containing subunit(gene name synonym) psi-mi:"MI:0007"(anti tag coimmunoprecipitation) Chu et al. (2007) imex:IM-28442|pubmed:17360195 taxid:9606(human)|taxid:9606(Homo sapiens) taxid:9606(human)|taxid:9606(Homo sapiens) psi-mi:"MI:0915"(physical association) psi-mi:"MI:0486"(UniProt) intact:EBI-26362347|imex:IM-28442-1 - - psi-mi:"MI:0499"(unspecified role) psi-mi:"MI:0499"(unspecified role) psi-mi:"MI:0496"(bait) psi-mi:"MI:0498"(prey) psi-mi:"MI:0326"(protein) psi-mi:"MI:0326"(protein) refseq:NP_071415.1|refseq:XP_005248624.1|ensembl:ENSG00000131844(gene)|ensembl:ENST00000340941(transcript)|go:"GO:0002169"(3-methylcrotonyl-CoA carboxylase complex, mitochondrial)|go:"GO:0004485"(methylcrotonoyl-CoA carboxylase activity)|go:"GO:0005524"(ATP binding)|go:"GO:0005739"(mitochondrion)|go:"GO:0005759"(mitochondrial matrix)|go:"GO:0005829"(cytosol)|go:"GO:0006552"(leucine catabolic process)|go:"GO:0015936"(coenzyme A metabolic process)|go:"GO:1905202"(methylcrotonoyl-CoA carboxylase complex)|interpro:IPR011762(Acetyl-coenzyme A carboxyltransferase, N-terminal)|interpro:IPR011763(Acetyl-coenzyme A carboxyltransferase, C-terminal)|interpro:IPR029045|interpro:IPR034733|mint:Q9HCC0|reactome:R-HSA-196780|reactome:R-HSA-3371599|reactome:R-HSA-70895 refseq:NP_064551.3|refseq:NP_001280202.1|interpro:IPR005481(Carbamoyl phosphate synthase, large subunit, N-terminal)|interpro:IPR005482(Biotin carboxylase, C-terminal)|interpro:IPR011053(Single hybrid motif)|interpro:IPR011054(Rudiment single hybrid motif)|interpro:IPR011761(ATP-grasp fold)|interpro:IPR011764(Biotin carboxylation region)|interpro:IPR016185(PreATP-grasp-like fold)|mint:Q96RQ3|rcsb pdb:2EJM|reactome:R-HSA-196780|reactome:R-HSA-3371599|reactome:R-HSA-70895|ensembl:ENSG00000078070(gene)|ensembl:ENST00000265594(transcript)|go:"GO:0002169"(3-methylcrotonyl-CoA carboxylase complex, mitochondrial)|go:"GO:0004075"(biotin carboxylase activity)|go:"GO:0004485"(methylcrotonoyl-CoA carboxylase activity)|go:"GO:0005524"(ATP binding)|go:"GO:0005739"(mitochondrion)|go:"GO:0005759"(mitochondrial matrix)|go:"GO:0005829"(cytosol)|go:"GO:0006552"(leucine catabolic process)|go:"GO:0006768"(biotin metabolic process)|go:"GO:0009374"(biotin binding)|go:"GO:0016421"(CoA carboxylase activity)|go:"GO:0046872"(metal ion binding)|go:"GO:1905202"(methylcrotonoyl-CoA carboxylase complex)|interpro:IPR000089(Biotin/lipoyl attachment)|interpro:IPR001882(Biotin-binding site)|interpro:IPR005479(Carbamoyl phosphate synthetase, large subunit, ATP-binding) - - - figure legend:Fig. 2A, 2B|full coverage:Only protein-protein interactions|curation depth:imex curation taxid:7108(spofr-sf_9)|taxid:7108(Spodoptera frugiperda insect cells) - 2020/09/30 2020/09/30 rogid:5NkqDKqdX1wHsbqXW7v5+/Gld109606 rogid:obze7v7i2yTIIcJjd7laIriNFg89606 rigid:5LYc8UtFdJC1Mazoe9uX+LnAbOg false myc tag:c-c flag tag:c-c - - psi-mi:"MI:0816"(molecular weight estimation by staining)|psi-mi:"MI:0705"(anti tag western blot) psi-mi:"MI:0816"(molecular weight estimation by staining)|psi-mi:"MI:0705"(anti tag western blot) uniprotkb:Q9HCC0 uniprotkb:Q96RQ3 intact:EBI-2211296|uniprotkb:A6NIY9|uniprotkb:Q96C27|uniprotkb:Q9Y4L7|ensembl:ENSP00000343657 intact:EBI-2211703|uniprotkb:Q59ES4|uniprotkb:Q9H959|uniprotkb:Q9NS97|ensembl:ENSP00000265594 psi-mi:mccb_human(display_long)|uniprotkb:MCCC2(gene name)|psi-mi:MCCC2(display_short)|uniprotkb:MCCB(gene name synonym)|uniprotkb:"3-methylcrotonyl-CoA:carbon dioxide ligase subunit beta"(gene name synonym)|uniprotkb:3-methylcrotonyl-CoA carboxylase non-biotin-containing subunit(gene name synonym)|uniprotkb:3-methylcrotonyl-CoA carboxylase 2(gene name synonym) psi-mi:mcca_human(display_long)|uniprotkb:MCCC1(gene name)|psi-mi:MCCC1(display_short)|uniprotkb:MCCA(gene name synonym)|uniprotkb:3-methylcrotonyl-CoA carboxylase 1(gene name synonym)|uniprotkb:"3-methylcrotonyl-CoA:carbon dioxide ligase subunit alpha"(gene name synonym)|uniprotkb:3-methylcrotonyl-CoA carboxylase biotin-containing subunit(gene name synonym) psi-mi:"MI:0007"(anti tag coimmunoprecipitation) Chu et al. (2007) imex:IM-28442|pubmed:17360195 taxid:9606(human)|taxid:9606(Homo sapiens) taxid:9606(human)|taxid:9606(Homo sapiens) psi-mi:"MI:0915"(physical association) psi-mi:"MI:0486"(UniProt) intact:EBI-26362424|imex:IM-28442-3 - - psi-mi:"MI:0499"(unspecified role) psi-mi:"MI:0499"(unspecified role) psi-mi:"MI:0498"(prey) psi-mi:"MI:0496"(bait) psi-mi:"MI:0326"(protein) psi-mi:"MI:0326"(protein) refseq:NP_071415.1|refseq:XP_005248624.1|ensembl:ENSG00000131844(gene)|ensembl:ENST00000340941(transcript)|go:"GO:0002169"(3-methylcrotonyl-CoA carboxylase complex, mitochondrial)|go:"GO:0004485"(methylcrotonoyl-CoA carboxylase activity)|go:"GO:0005524"(ATP binding)|go:"GO:0005739"(mitochondrion)|go:"GO:0005759"(mitochondrial matrix)|go:"GO:0005829"(cytosol)|go:"GO:0006552"(leucine catabolic process)|go:"GO:0015936"(coenzyme A metabolic process)|go:"GO:1905202"(methylcrotonoyl-CoA carboxylase complex)|interpro:IPR011762(Acetyl-coenzyme A carboxyltransferase, N-terminal)|interpro:IPR011763(Acetyl-coenzyme A carboxyltransferase, C-terminal)|interpro:IPR029045|interpro:IPR034733|mint:Q9HCC0|reactome:R-HSA-196780|reactome:R-HSA-3371599|reactome:R-HSA-70895 refseq:NP_064551.3|refseq:NP_001280202.1|interpro:IPR005481(Carbamoyl phosphate synthase, large subunit, N-terminal)|interpro:IPR005482(Biotin carboxylase, C-terminal)|interpro:IPR011053(Single hybrid motif)|interpro:IPR011054(Rudiment single hybrid motif)|interpro:IPR011761(ATP-grasp fold)|interpro:IPR011764(Biotin carboxylation region)|interpro:IPR016185(PreATP-grasp-like fold)|mint:Q96RQ3|rcsb pdb:2EJM|reactome:R-HSA-196780|reactome:R-HSA-3371599|reactome:R-HSA-70895|ensembl:ENSG00000078070(gene)|ensembl:ENST00000265594(transcript)|go:"GO:0002169"(3-methylcrotonyl-CoA carboxylase complex, mitochondrial)|go:"GO:0004075"(biotin carboxylase activity)|go:"GO:0004485"(methylcrotonoyl-CoA carboxylase activity)|go:"GO:0005524"(ATP binding)|go:"GO:0005739"(mitochondrion)|go:"GO:0005759"(mitochondrial matrix)|go:"GO:0005829"(cytosol)|go:"GO:0006552"(leucine catabolic process)|go:"GO:0006768"(biotin metabolic process)|go:"GO:0009374"(biotin binding)|go:"GO:0016421"(CoA carboxylase activity)|go:"GO:0046872"(metal ion binding)|go:"GO:1905202"(methylcrotonoyl-CoA carboxylase complex)|interpro:IPR000089(Biotin/lipoyl attachment)|interpro:IPR001882(Biotin-binding site)|interpro:IPR005479(Carbamoyl phosphate synthetase, large subunit, ATP-binding) - - - figure legend:Fig. 4A, 4B|full coverage:Only protein-protein interactions|curation depth:imex curation taxid:7108(spofr-sf_9)|taxid:7108(Spodoptera frugiperda insect cells) - 2020/09/30 2020/09/30 rogid:5NkqDKqdX1wHsbqXW7v5+/Gld109606 rogid:obze7v7i2yTIIcJjd7laIriNFg89606 rigid:5LYc8UtFdJC1Mazoe9uX+LnAbOg false myc tag:c-c flag tag:c-c - - psi-mi:"MI:0816"(molecular weight estimation by staining)|psi-mi:"MI:0705"(anti tag western blot) psi-mi:"MI:0816"(molecular weight estimation by staining)|psi-mi:"MI:0705"(anti tag western blot) uniprotkb:Q9HCC0 uniprotkb:Q96RQ3 intact:EBI-2211296|uniprotkb:A6NIY9|uniprotkb:Q96C27|uniprotkb:Q9Y4L7|ensembl:ENSP00000343657 intact:EBI-2211703|uniprotkb:Q59ES4|uniprotkb:Q9H959|uniprotkb:Q9NS97|ensembl:ENSP00000265594 psi-mi:mccb_human(display_long)|uniprotkb:MCCC2(gene name)|psi-mi:MCCC2(display_short)|uniprotkb:MCCB(gene name synonym)|uniprotkb:"3-methylcrotonyl-CoA:carbon dioxide ligase subunit beta"(gene name synonym)|uniprotkb:3-methylcrotonyl-CoA carboxylase non-biotin-containing subunit(gene name synonym)|uniprotkb:3-methylcrotonyl-CoA carboxylase 2(gene name synonym) psi-mi:mcca_human(display_long)|uniprotkb:MCCC1(gene name)|psi-mi:MCCC1(display_short)|uniprotkb:MCCA(gene name synonym)|uniprotkb:3-methylcrotonyl-CoA carboxylase 1(gene name synonym)|uniprotkb:"3-methylcrotonyl-CoA:carbon dioxide ligase subunit alpha"(gene name synonym)|uniprotkb:3-methylcrotonyl-CoA carboxylase biotin-containing subunit(gene name synonym) psi-mi:"MI:0071"(molecular sieving) Chu et al. (2007) imex:IM-28442|pubmed:17360195 taxid:9606(human)|taxid:9606(Homo sapiens) taxid:9606(human)|taxid:9606(Homo sapiens) psi-mi:"MI:0407"(direct interaction) psi-mi:"MI:0486"(UniProt) intact:EBI-26362438|imex:IM-28442-5 - - psi-mi:"MI:0499"(unspecified role) psi-mi:"MI:0499"(unspecified role) psi-mi:"MI:0497"(neutral component) psi-mi:"MI:0497"(neutral component) psi-mi:"MI:0326"(protein) psi-mi:"MI:0326"(protein) refseq:NP_071415.1|refseq:XP_005248624.1|ensembl:ENSG00000131844(gene)|ensembl:ENST00000340941(transcript)|go:"GO:0002169"(3-methylcrotonyl-CoA carboxylase complex, mitochondrial)|go:"GO:0004485"(methylcrotonoyl-CoA carboxylase activity)|go:"GO:0005524"(ATP binding)|go:"GO:0005739"(mitochondrion)|go:"GO:0005759"(mitochondrial matrix)|go:"GO:0005829"(cytosol)|go:"GO:0006552"(leucine catabolic process)|go:"GO:0015936"(coenzyme A metabolic process)|go:"GO:1905202"(methylcrotonoyl-CoA carboxylase complex)|interpro:IPR011762(Acetyl-coenzyme A carboxyltransferase, N-terminal)|interpro:IPR011763(Acetyl-coenzyme A carboxyltransferase, C-terminal)|interpro:IPR029045|interpro:IPR034733|mint:Q9HCC0|reactome:R-HSA-196780|reactome:R-HSA-3371599|reactome:R-HSA-70895 refseq:NP_064551.3|refseq:NP_001280202.1|interpro:IPR005481(Carbamoyl phosphate synthase, large subunit, N-terminal)|interpro:IPR005482(Biotin carboxylase, C-terminal)|interpro:IPR011053(Single hybrid motif)|interpro:IPR011054(Rudiment single hybrid motif)|interpro:IPR011761(ATP-grasp fold)|interpro:IPR011764(Biotin carboxylation region)|interpro:IPR016185(PreATP-grasp-like fold)|mint:Q96RQ3|rcsb pdb:2EJM|reactome:R-HSA-196780|reactome:R-HSA-3371599|reactome:R-HSA-70895|ensembl:ENSG00000078070(gene)|ensembl:ENST00000265594(transcript)|go:"GO:0002169"(3-methylcrotonyl-CoA carboxylase complex, mitochondrial)|go:"GO:0004075"(biotin carboxylase activity)|go:"GO:0004485"(methylcrotonoyl-CoA carboxylase activity)|go:"GO:0005524"(ATP binding)|go:"GO:0005739"(mitochondrion)|go:"GO:0005759"(mitochondrial matrix)|go:"GO:0005829"(cytosol)|go:"GO:0006552"(leucine catabolic process)|go:"GO:0006768"(biotin metabolic process)|go:"GO:0009374"(biotin binding)|go:"GO:0016421"(CoA carboxylase activity)|go:"GO:0046872"(metal ion binding)|go:"GO:1905202"(methylcrotonoyl-CoA carboxylase complex)|interpro:IPR000089(Biotin/lipoyl attachment)|interpro:IPR001882(Biotin-binding site)|interpro:IPR005479(Carbamoyl phosphate synthetase, large subunit, ATP-binding) - - - figure legend:Fig. 6|full coverage:Only protein-protein interactions|curation depth:imex curation taxid:-1(in vitro)|taxid:-1(In vitro) - 2020/09/30 2020/10/22 rogid:5NkqDKqdX1wHsbqXW7v5+/Gld109606 rogid:obze7v7i2yTIIcJjd7laIriNFg89606 rigid:5LYc8UtFdJC1Mazoe9uX+LnAbOg false myc tag:c-c flag tag:c-c - - psi-mi:"MI:0113"(western blot) psi-mi:"MI:0113"(western blot) chebi:"CHEBI:15486" reactome:R-HSA-3323185 intact:EBI-26362518|chebi:"CHEBI:27728"|chebi:"CHEBI:1599"|chebi:"CHEBI:11853"|chebi:"CHEBI:11858"|chebi:"CHEBI:23802"|chebi:"CHEBI:20128"|chebi:"CHEBI:4615" intact:EBI-26362447|reactome:R-HSA-3323135|intenz:6.4.1.4|rhea:"RHEA:13589"|reactome:R-HSA-70770 psi-mi:3-methylbut-2-enoyl-coa(display_short)|psi-mi:"CHEBI:15486"(display_long)|intact:3,3-dimethacrylyl-CoA(synonym)|intact:3,3-dimethacrylyl-coenzyme A(synonym)|intact:3-Methylbut-2-enoyl-CoA(synonym)|intact:3-Methylcrotonoyl-CoA(synonym)|intact:3-Methylcrotonyl-CoA(synonym)|intact:beta,beta-dimethacrylyl-CoA(synonym)|intact:beta,beta-dimethacrylyl-coenzyme A(synonym)|intact:Dimethylacryloyl-CoA(synonym)|intact:DMA-CoA(synonym)|intact:"S-(3-Methyl-crotonoyl)-coenzym-A"(synonym)|intact:"S-(3-methylcrotonoyl)-coenzyme-A"(synonym)|intact:"3'-phosphoadenosine 5'-{3-[(3R)-3-hydroxy-2,2-dimethyl-4-{[3-({2-[(3-methylbut-2-enoyl)sulfanyl]ethyl}amino)-3-oxopropyl]amino}-4-oxobutyl] dihydrogen diphosphate}"(iupac name) psi-mi:mccc2-mccc1-1(display_short)|psi-mi:R-HSA-3323185(display_long)|intact:3-methylcrotonyl-CoA carboxylase 1 complex(complex synonym)|intact:"3-methylcrotonyl-CoA:carbon dioxide ligase complex"(complex synonym)|intact:MCCC complex(complex synonym)|intact:Mitochondrial methylcrotonoyl-CoA carboxylase complex(complex synonym)|intact:"MCCC1:MCCC2"(complex systematic name)|intact:Mitochondrial methylcrotonyl-CoA carboxylase complex(complex recommended name) psi-mi:"MI:0415"(enzymatic study) Chu et al. (2007) imex:IM-28442|pubmed:17360195 taxid:-2(chemical synthesis)|taxid:-2("Chemical synthesis (Chemical synthesis)") taxid:9606(human)|taxid:9606(Homo sapiens) psi-mi:"MI:0414"(enzymatic reaction) psi-mi:"MI:0486"(UniProt) intact:EBI-26362542|imex:IM-28442-7 - - psi-mi:"MI:0502"(enzyme target) psi-mi:"MI:0501"(enzyme) psi-mi:"MI:0499"(unspecified role) psi-mi:"MI:0499"(unspecified role) psi-mi:"MI:0328"(small molecule) psi-mi:"MI:1302"(stable complex) - go:"GO:0004485"(methylcrotonoyl-CoA carboxylase activity)|go:"GO:0009083"(branched-chain amino acid catabolic process)|intact:EBI-26362438(exp-evidence)|complex portal:CPX-6236(complex-primary)|evidence ontology:"ECO:0000353"|pubmed:17360195(see-also)|go:"GO:0002169"(3-methylcrotonyl-CoA carboxylase complex, mitochondrial)|pubmed:22869039(see-also)|go:"GO:0005524"(ATP binding)|efo:"Orphanet:6"(see-also) go:"GO:0004485"(methylcrotonoyl-CoA carboxylase activity) - curated-complex:Catalyzes the conversion of 3-methylcrotonyl-CoA to 3-methylglutaconyl-CoA, a critical step for leucine and isovaleric acid catabolism. The biotin carboxylase component catalyzes the Mg-ATP-dependent carboxylation of the N1' atom of the biotin cofactor, using bicarbonate as the CO2 donor. The carboxyltransferase component then catalyzes the CO2 transfer from carboxybiotin to the gamma carbon of the alpha-beta unsaturated acid, 3-methylcrotonyl-CoA.|complex-properties:Molecular weight 500-800 kDa|complex-assembly:Heterododecamer|disease:"3-methylcrotonoyl-CoA carboxylase deficiency (MCCD) [Orphanet:6]: an autosomal recessive inherited disorder of leucine metabolism characterized by a highly variable clinical picture ranging from metabolic crisis in infancy to asymptomatic adults. There is a characteristic organic aciduria with massive excretion of 3- hydroxyisovaleric acid and 3-methylcrotonylglycine, usually in combination with a severe secondary carnitine deficiency." figure legend:Fig. 2C, Fig 3|full coverage:Only protein-protein interactions|curation depth:imex curation taxid:-1(in vitro)|taxid:-1(In vitro) km:7.4x10^-5(molar) 2020/09/30 2020/09/30 - - - false - - - - psi-mi:"MI:0396"(predetermined participant) psi-mi:"MI:0396"(predetermined participant)