Figure 1.

Mitochondrial catabolism of propionyl-CoA to succinyl-CoA

Propionic acidemia (PA) is caused by mutations in the PCCA or PCCB gene, which encode for the respective subunits of propionyl-CoA carboxylase (PCC). Enzymes downstream of PCC in the metabolism of propionyl-CoA to succinyl-CoA, a Krebs cycle intermediate. Other enzymes include D-methylmalonyl-CoA epimerase (MCEE) and methylmalonyl-CoA mutase (MMUT). The figure was created with BioRender.