The Propionyl-CoA Carboxylase (PCC) Project

Propionyl-CoA carboxylase (PCC) catalyzes the biotin-dependent carboxylation of propionyl-CoA to produce D-methylmalonyl-CoA. PCC is essential for the catabolism of the amino acids Thr, Val, Ile and Met, cholesterol, and fatty acids with an odd number of carbon atoms.

Deficiencies of PCC activity in humans are linked to the disease propionic acidemia (PA), an autosomal recessive disorder that can be fatal in infants.

PCC holoenzyme is a 750-kD a6b6 dodecamer. The a subunit is known to contain the biotin carboxylase (BC) and biotin carboxyl carrier protein (BCCP) domains, and the b subunit supplies the carboxyltransferase (CT) activity.

Major findings from this project

Publications from this project

Funding for this project

© copyright 2010-2017, Liang Tong.