Isolated mitochondrial respiratory chain complex IV deficiency (Q102292)

This refers to isolated coupled electron transfer between an electron donor (such as NADH) and an electron acceptor (such as O2) with the transfer of H+ ions (protons) across a membrane. This diagnosis is with deficiency in Complex IV, with four electrons being removed from four molecules of cytochrome c and transferred to molecular oxygen (O2), producing two molecules of water.