NQO1 and Leber hereditary optic neuropathy: Idebenone, a CoQ10 analog, is the first drug approved in Europe for the treatment of LHON.45 It is reduced by cytosolic NAD(P)H oxidoreductase I (NQO1)747 and bypasses LHON-associated complex I dysfunction, restoring mitochondrial function by shuttling electrons directly from the cytoplasm to complex III.