Figure 2:

Proposed mechanism of cardiotoxicity in propionic acidemia. A deficiency in propionyl-CoA carboxylase results in accumulation of propionyl-CoA and other metabolites. These compounds mediate oxidative damage, compromising the generation of energy-rich adenosine triphosphate (ATP) via oxidative phosphorylation. The resulting cardiotoxicity is manifest in the form of ventricular dysfunction or predisposition to arrhythmogenesis. Note: ADP = adenosine diphosphate, CoA = coezyme A, e⁻ = electron, H⁺ = hydrogen ion, H₂O = water, O₂ = molecular oxygen.