LMPD Database

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LMP000403

UniProt Annotations

Entry Information
Gene Namepropionyl CoA carboxylase, alpha polypeptide
Protein EntryPCCA_HUMAN
UniProt IDP05165
SpeciesHuman
Comments
Comment typeDescription
Alternative ProductsEvent=Alternative splicing; Named isoforms=3; Name=1; IsoId=P05165-1; Sequence=Displayed; Name=2; IsoId=P05165-2; Sequence=VSP_039857; Name=3; IsoId=P05165-3; Sequence=VSP_044458;
Catalytic ActivityATP + propanoyl-CoA + HCO(3)(-) = ADP + phosphate + (S)-methylmalonyl-CoA.
CofactorName=biotin; Xref=ChEBI
DiseasePropionic acidemia type I (PA-1) [MIM
PathwayMetabolic intermediate metabolism; propanoyl-CoA degradation; succinyl-CoA from propanoyl-CoA
Sequence CautionSequence=AAA60035.1; Type=Frameshift; Positions=374, 378, 380, 395; Evidence= ; Sequence=AAH00140.1; Type=Erroneous initiation; Note=Translation N-terminally extended.; Evidence= ; Sequence=AAK61392.1; Type=Erroneous initiation; Note=Translation N-terminally extended.; Evidence= ; Sequence=CAA32763.1; Type=Frameshift; Positions=23; Evidence= ;
SimilarityContains 1 ATP-grasp domain. {ECO
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.