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PCCA mAb

The protein encoded by this gene is the alpha subunit of the heterodimeric mitochondrial enzyme Propionyl-CoA carboxylase. PCCA encodes the biotin-binding region of this enzyme. Mutations in either PCCA or PCCB (encoding the beta subunit) lead to an enzyme deficiency resulting in propionic acidemia. Multiple transcript variants encoding different isoforms have been found for this gene

Alternative Name(s)

PCCAalpha polypeptide; mitochondrial; pccA complementation group; PCCase alpha subunit; PCCase subunit alpha; propanoyl-CoA:carbon dioxide ligase alpha subunit; Propanoyl-CoA:carbon dioxide ligase subunit alpha; propionyl CoA carboxylase, alpha polypeptide; propionyl Coenzyme A carboxylase; propionyl Coenzyme A carboxylase, alpha polypeptide; propionyl-CoA carboxylase alpha chain; Propionyl-CoA carboxylase alpha chain, mitochondrial;PCCA

Application

WB, IF

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UNIprot

P05165

clonality

Monoclonal

reactivity

Human,Mouse,Rat

Research area

Fatty Acid Metabolism

source

Mouse

storage

Store at -20℃, stable for one year from the date of shipment.

PCCA mAb

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