Protein: Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial (MCCase subunit alpha) (EC 6.4.1.4) (3-methylcrotonyl-CoA carboxylase 1) (3-methylcrotonyl-CoA carboxylase biotin-containing subunit) (3-methylcrotonyl-CoA:carbon dioxide ligase subunit alpha) (Q99MR8)
Organism: Mus musculus (Mouse)
Function: Biotin-attachment subunit of the 3-methylcrotonyl-CoA carboxylase, an enzyme that catalyzes the conversion of 3-methylcrotonyl-CoA to 3-methylglutaconyl-CoA, a critical step for leucine and isovaleric acid catabolism.
Subcellular location: Mitochondrion matrix {ECO:0000250|UniProtKB:Q96RQ3}.
Sequence: MAAAALLAAVDRNQLRRVPILLLQPREWAWKLRTMKYGTTPGGSITKVLIANRGEIACRVIRTAKKMGVQSVAVYSEADRNSMHVDMADEAYSIGPAPSQQSYLAMEKIIQVAKSSAAQAIHPGYGFLSENMEFAELCKQEGIIFIGPPSSAIRDMGIKSTSKSIMAAAGVPVVEGYHGKDQSDQCLREHAGKIGYPVMIKAVRGGGGKGMRIVRSEREFQEQLESARREAKKSFNDDAMLIEKFVDTPRHVEVQVFGDHHGNAVYLFERDCSVQRRHQKIIEEAPAPGINPEVRRKLGEAAVRAAKAVKYVGAGTVEFIMDSRHNFYFMEMNTRLQVEHPVTEMITGTDLVEWQLRIAAGEKIPLSQEEIPLQGHAFEARIYAEDPDNNFMPGAGPLVHLSTPSADMSTRIETGVRQGDEVSVHYDPMIAKLVVWASDRQSALSKLRYCLHQYNIVGLRSNVDFLLRLSGHPEFEAGNVHTDFIPQHHKDLLPSHSTIAKESVCQAALGLILKEKEMTSAFKLHTQDQFSPFSFSSGRRLNISYTRNMTLRSGKSDIVIAVTYNRDGSYDMQIDNKSFRVLGDLSSEDGCTYLKSSINGVARKSKFILLDNTVHLFSMEGSIEVGIPVPKYLSPVSAEGAQGGTIAPMTGTIEKVFVKAGDRVKAGDSLMVMIAMKMEHTIKAPKDGRIKKVFFSEGAQANRHAPLVEFEEEESDK
InterPro domains: - IPR005481: Biotin carboxylase-like, N-terminal domain (domain) [45-153]
- IPR016185: Pre-ATP-grasp domain superfamily (homologous_superfamily) [42-156]
- IPR000089: Biotin/lipoyl attachment (domain) [622-711]
- IPR005482: Biotin carboxylase, C-terminal (domain) [379-486]
- IPR011761: ATP-grasp fold (domain) [163-360]
- IPR005479: Carbamoyl phosphate synthase, ATP-binding domain (domain) [159-365]
- IPR050856: Biotin-dependent Carboxylase Complex (family) [41-713]
- IPR011764: Biotin carboxylation domain (domain) [44-490]
- IPR001882: Biotin-binding site (binding_site) [667-684]
- IPR011054: Rudiment single hybrid motif (homologous_superfamily) [374-491]
- IPR011053: Single hybrid motif (homologous_superfamily) [635-711]
Interaction partners: - Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial
- Propionyl-CoA carboxylase beta chain, mitochondrial
- Isovaleryl-CoA dehydrogenase, mitochondrial
- Enoyl-CoA hydratase, mitochondrial
- Trifunctional enzyme subunit alpha, mitochondrial
- Peroxisomal bifunctional enzyme
- Medium-chain specific acyl-CoA dehydrogenase, mitochondrial
- Methylglutaconyl-CoA hydratase, mitochondrial
- Biotin--protein ligase
- Propionyl-CoA carboxylase alpha chain, mitochondrial
Gene Ontology annotations:
Molecular Function (MF): GO:0003674 (molecular function), GO:0003824 (catalytic activity), GO:0016874 (ligase activity), GO:0016885 (ligase activity, forming carbon-carbon bonds), GO:0016421 (CoA carboxylase activity)
Biological Process (BP): GO:0008150 (biological process), GO:0008152 (metabolic process), GO:0009987 (cellular process), GO:0044237 (cellular metabolic process), GO:0071704 (organic substance metabolic process), GO:0044281 (small molecule metabolic process), GO:0009056 (catabolic process), GO:0044238 (primary metabolic process), GO:0044255 (cellular lipid metabolic process), GO:0006629 (lipid metabolic process), GO:0044282 (small molecule catabolic process), GO:1901575 (organic substance catabolic process), GO:0044248 (cellular catabolic process), GO:0006082 (organic acid metabolic process), GO:0006631 (fatty acid metabolic process), GO:0016054 (organic acid catabolic process), GO:0044242 (cellular lipid catabolic process), GO:0043436 (oxoacid metabolic process), GO:0016042 (lipid catabolic process), GO:0019752 (carboxylic acid metabolic process), GO:0046395 (carboxylic acid catabolic process), GO:0046459 (short-chain fatty acid metabolic process), GO:0009062 (fatty acid catabolic process), GO:0072329 (monocarboxylic acid catabolic process), GO:0032787 (monocarboxylic acid metabolic process), GO:0019626 (short-chain fatty acid catabolic process)
Cellular Component (CC): GO:0005575 (cellular component), GO:0110165 (cellular anatomical entity), GO:0005622 (intracellular anatomical structure), GO:0043226 (organelle), GO:0005737 (cytoplasm), GO:0043229 (intracellular organelle), GO:0005739 (mitochondrion), GO:0043227 (membrane-bounded organelle), GO:0043231 (intracellular membrane-bounded organelle)