Mitochondrial ATP synthase is composed of two multi-subunit complexes that utilize an inner membrane electrochemical gradient to catalyze the synthesis of ATP during oxidative phosphorylation. The two multi-subunit complexes are designated F1 and F0, the former of which comprises the soluble catalytic core and the latter of which comprises the membrane-spanning proton channel of ATP synthase. F1 consists of five distinct subunits, designated ATP5A, ATP5B, ATP5C1, ATP5D and ATP5E, while F0 co