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

Publication

PubMed ID:
11170888 11401427 11406611 11181649 14702039 15489334 16023992 17360195 21269460 24275569 25944712 16010683 17968484 21071250 22150417 22264772 22642865