LMPD Database

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LMP000406

UniProt Annotations

Entry Information
Gene Namepropionyl CoA carboxylase, beta polypeptide
Protein EntryPCCB_HUMAN
UniProt IDP05166
SpeciesHuman
Comments
Comment typeDescription
Alternative ProductsEvent=Alternative splicing; Named isoforms=2; Name=1; IsoId=P05166-1; Sequence=Displayed; Name=2; IsoId=P05166-2; Sequence=VSP_042568; Note=No experimental confirmation available.;
Catalytic ActivityATP + propanoyl-CoA + HCO(3)(-) = ADP + phosphate + (S)-methylmalonyl-CoA.
DiseasePropionic acidemia type II (PA-2) [MIM
PathwayMetabolic intermediate metabolism; propanoyl-CoA degradation; succinyl-CoA from propanoyl-CoA
SimilarityBelongs to the AccD/PCCB family.
SimilarityContains 1 carboxyltransferase domain.
Subcellular LocationMitochondrion matrix .
SubunitProbably a dodecamer composed of six biotin-containing alpha subunits and six beta subunits.