Blue Native polyacrylamide gel from a.) muscle, b.) liver and c.) heart tissue showing activity staining for complexes I, II, IV and V as indicated. A) Little complex I or IV are appreciated in muscle, with incomplete complex V assembly as evidenced by lower molecular weight bands but with preserved complex II. B) Increased activity of complex II and incomplete complex V assembly in liver. C) Low levels of complex I and IV, with incomplete complex V assembly in heart. Ctrl- control tissue, HSD10- patient tissue.