propionyl-CoA + CO2 + ATP <=> D-methylmalonyl-CoA + ADP + orthophosphate EC Number: 6.4.1.3 EC Number: ase ISBN0079130356 Propionyl CoA carboxylase in the mitochondrial matrix catalyzes the reaction of propionyl-CoA, CO2, and ATP to form D-methylmalonyl-CoA, ADP, and orthophosphate. The active form of the enzyme is a heteromultimer, probably consisting of six alpha subunits each bound to a biotin molecule and six beta subunits (Kaziro et al. 1961; Kalousek et al. 1980; Fenton et al. 2001). Both alpha and beta subunits are posttranslationally modified to remove amino-terminal mitochondrial import sequences (Stadler et al. 2005). Pubmed13752080 Pubmed16023992 Pubmed6765947 Reactome Database ID Release 4371031 Reactome, http://www.reactome.org ReactomeREACT_580