propionyl CoA carboxylase-beta chain (PCCB)

Function

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

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

Structure

• probably a dodecamer composed of six biotin-containing alpha subunits & six beta subunits
• belongs to the accD/PCCB family
• contains 1 carboxyltransferase domain

Compartment

mitochondrial matrix

Pathology

-defects in PCCB are the cause of propionic acidemia type 2

More general terms

• protein subunit

Component of

• propionyl CoA carboxylase

Database

• UniProt: http://www.uniprot.org/uniprot/P05166.html
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:5096
• OMIM: https://mirror.omim.org/entry/232050
• OMIM: https://mirror.omim.org/entry/606054