Though it had no counterpart in human disease, rescue of an animal model of complex I deficiency induced by the NDUFA1 ribozyme with a gene that neutralizes reactive oxygen species (mitochondrial superoxide dismutase) proved that in that model system, suppression of reactive oxygen species inhibited death of retinal ganglion cells, a phenomenon that we showed in the LHON animal model is also involved in the pathogenesis of neurodegeneration caused by the mutant human ND4 complex I subunit gene [24]. Here, NDUFA1 is linked to Leber hereditary optic neuropathy.