Propionyl-CoA carboxylase

Function

Propionyl-CoA carboxylase (PCC) is a biotin-dependent enzyme that catalyzes the conversion of propionyl-CoA to D-methylmalonyl-CoA^[1]. PCC is composed of two subunits - α and β. The β subunit is the carboxyltransferase subunit. See also 3-Hydroxypropionate_bicycle.

Disease

Mutations in the β subunit cause PCC deficiency resulting in the metabolic disorder - propionic acidemia^[2].

Relevance

Structural highlights

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