One final advantage is that, unlike a mouse model with a mitochondrial inheritance pattern, the Vglut2-Cre;ndufs4loxP/loxP mouse line described here may be used to generate control littermates for experiments (e.g. half of the progeny from the cross of a Vglut2-Cre;ndufs4loxP/loxP male to a ndufs4loxP/loxP female will be ndufs4loxP/loxP mice that do not develop optic atrophy). The gene discussed is SLC17A6; the disease is Leber hereditary optic neuropathy.