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.
Date
2022-02
Language
eng
Format
video/mp4
Type
Image/MovingImage
Source
2022 North American Neuro-Ophthalmology Society Annual Meeting
Relation is Part of
NANOS Annual Meeting 2022: Scientific Platform III