Continuous Hypoxia Reduces Retinal Ganglion Cell Degeneration in a Mouse Model of Mitochondrial Optic Neuropathy

Primary optic atrophy resulting from death of retinal ganglion cells (RGCs) is the most prominent ocular manifestation of mitochondrial disease. We have recently developed a genetic mouse model of rapidly-progressive mitochondrial optic neuropathy in which RGCs experience severe dysfunction of mitochondrial respiratory complex I due to conditional deletion of the accessory subunit ndufs4 within RGCs.1 While continuous hypoxia was previously shown to prolong the lifespan of the Leigh syndrome model ndufs4 knockout mouse bearing a global deletion of the gene, any effect of hypoxia on RGC survival has not been reported.