propionyl CoA carboxylase-alpha chain (PCCA)

Function

• catalytic subunit of propionyl CoA carboxylase
• carbohydrate metabolism; propanoate metabolism

ATP + propanoyl-CoA + HCO3-
              <-->
ADP + phosphate + (S)-methylmalonyl-CoA

Cofactor: biotin

Structure

• probably a dodecamer composed of six biotin-containing alpha subunits & six beta subunits
• contains 1 ATP-grasp domain
• contains 1 biotin carboxylation domain
• contains 1 biotinyl-binding domain

Compartment

mitochondrial matrix

Pathology

• defects in PCCA are the cause of propionic acidemia type 1

More general terms

• enzyme catalytic subunit
• phosphoprotein

Component of

• propionyl CoA carboxylase

Database

• UniProt: http://www.uniprot.org/uniprot/P05165.html
• OMIM: https://mirror.omim.org/entry/232000
• OMIM: https://mirror.omim.org/entry/606054