Isolated mitochondrial respiratory chain complex II deficiency (Q102297)

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 II, consisting of four protein subunits: SDHA, SDHB, SDHC, and SDHD. Other electron donors (e.g., fatty acids an glycerol 3-phosphate) also direct electrons into Q (via FAD).