Names & Taxonomy
- Uniprot ID:
- Q96RQ3
- Entry Name:
- MCCA_HUMAN
- Status:
- reviewed
- Protein Names:
- 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)
- Gene Names:
- MCCC1 MCCA
- Gene Names Primary:
- MCCC1
- Organism:
- Homo sapiens (Human)
Structure
- Length:
- 725
- Sequence:
- MAAASAVSVLLVAAERNRWHRLPSLLLPPRTWVWRQRTMKYTTATGRNITKVLIANRGEIACRVMRTAKKLGVQTVAVYSEADRNSMHVDMADEAYSIGPAPSQQSYLSMEKIIQVAKTSAAQAIHPGCGFLSENMEFAELCKQEGIIFIGPPPSAIRDMGIKSTSKSIMAAAGVPVVEGYHGEDQSDQCLKEHARRIGYPVMIKAVRGGGGKGMRIVRSEQEFQEQLESARREAKKSFNDDAMLIEKFVDTPRHVEVQVFGDHHGNAVYLFERDCSVQRRHQKIIEEAPAPGIKSEVRKKLGEAAVRAAKAVNYVGAGTVEFIMDSKHNFCFMEMNTRLQVEHPVTEMITGTDLVEWQLRIAAGEKIPLSQEEITLQGHAFEARIYAEDPSNNFMPVAGPLVHLSTPRADPSTRIETGVRQGDEVSVHYDPMIAKLVVWAADRQAALTKLRYSLRQYNIVGLHTNIDFLLNLSGHPEFEAGNVHTDFIPQHHKQLLLSRKAAAKESLCQAALGLILKEKAMTDTFTLQAHDQFSPFSSSSGRRLNISYTRNMTLKDGKNNVAIAVTYNHDGSYSMQIEDKTFQVLGNLYSEGDCTYLKCSVNGVASKAKLIILENTIYLFSKEGSIEIDIPVPKYLSSVSSQETQGGPLAPMTGTIEKVFVKAGDKVKAGDSLMVMIAMKMEHTIKSPKDGTVKKVFYREGAQANRHTPLVEFEEEESDKRESE
- Proteomes:
- UP000005640
Subcellular location
- Subcellular Location:
- Mitochondrion matrix
Function
- 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.
- Pathway:
- Amino-acid degradation; L-leucine degradation; (S)-3-hydroxy-3-methylglutaryl-CoA from 3-isovaleryl-CoA: step 2/3.
- Catalytic Activity:
- ATP + 3-methylcrotonoyl-CoA + HCO(3)(-) = ADP + phosphate + 3-methylglutaconyl-CoA.
- Cofactor:
- COFACTOR: Name=biotin; Xref=ChEBI:CHEBI:57586;
- Active Site:
- ACT_SITE 339 339
- Cross Reference Drug Bank:
- DB00121
- Gene Ontology Go:
- cytosol
mitochondrial inner membrane
mitochondrial matrix
mitochondrion
ATP binding
biotin binding
biotin carboxylase activity
metal ion binding
methylcrotonoyl-CoA carboxylase activity
biotin metabolic process
branched-chain amino acid catabolic process
cellular nitrogen compound metabolic process
leucine catabolic process
small molecule metabolic process
vitamin metabolic process
water-soluble vitamin metabolic process - Gene Ontology Biological Process:
- biotin metabolic process
branched-chain amino acid catabolic process
cellular nitrogen compound metabolic process
leucine catabolic process
small molecule metabolic process
vitamin metabolic process
water-soluble vitamin metabolic process - Gene Ontology Molecular Function:
- ATP binding
biotin binding
biotin carboxylase activity
metal ion binding
methylcrotonoyl-CoA carboxylase activity - Gene Ontology Cellular Component:
- cytosol
mitochondrial inner membrane
mitochondrial matrix
mitochondrion - Keywords:
- 3D-structure
ATP-binding
Acetylation
Biotin
Complete proteome
Direct protein sequencing
Disease mutation
Ligase
Mitochondrion
Nucleotide-binding
Polymorphism
Reference proteome
Transit peptide