LMPD Database

New search | Record Overview | Ontology/Pathway Information | Domain Information | UniProt Annotations | Related Proteins
LMP013636

UniProt Annotations

Entry Information
Gene Namepropionyl CoA carboxylase, alpha polypeptide
Protein EntryPCCA_RAT
UniProt IDP14882
SpeciesRat
Comments
Comment typeDescription
Catalytic ActivityATP + propanoyl-CoA + HCO(3)(-) = ADP + phosphate + (S)-methylmalonyl-CoA.
CofactorName=biotin; Xref=ChEBI:CHEBI:57586; Note=Biotin.;
DiseaseNote=Propionic acidemia due to recessively inherited deficiency of PCCase activity often causes life-threatening ketosis and acidosis.
PathwayMetabolic intermediate metabolism; propanoyl-CoA degradation; succinyl-CoA from propanoyl-CoA: step 1/3.
SimilarityContains 1 ATP-grasp domain. {ECO:0000255|PROSITE- ProRule:PRU00409}.
SimilarityContains 1 biotin carboxylation domain
SimilarityContains 1 biotinyl-binding domain
Subcellular LocationMitochondrion matrix.
SubunitProbably a dodecamer composed of six biotin-containing alpha subunits and six beta subunits.