Pcca - propionyl-Coenzyme A carboxylase, alpha polypeptide

Enables propionyl-CoA carboxylase activity. Predicted to act upstream of or within lipid catabolic process. Located in mitochondrion. Is expressed in several structures, including alimentary system; nervous system; orbito-sphenoid; sensory organ; and urinary system. Used to study propionic acidemia. Human ortholog(s) of this gene implicated in amino acid metabolic disorder and propionic acidemia. Orthologous to human PCCA (propionyl-CoA carboxylase subunit alpha). [provided by Alliance of Genome Resources, Apr 2022]