Leber’s hereditary optic neuropathy (LHON) is an inherited retinal degenerative disorder that causes a progressive loss in visual acuity, and eventual complete loss of vision. LHON is caused by mitochondrial DNA mutations which lead to retinal cell dysfunction. There are currently no available treatments for LHON, but patients often supplement with antioxidants in an attempt to manage disease onset.
Rotenone is a chemical agent that inhibits mitochondrial electron transport. Intravitreal injection of rotenone causes a rapid onset of localized ocular defects that recapitulate LHON disease in humans. This animal model can be used to test therapeutic intervention on clinical outcomes. Following induction of the disease, visual acuity is monitored by means of optokinetic tracking (OKT), and retinal cell function is monitored by electroretinography (ERG). Both measures are compared to baseline readings. Terminal histology measures retinal degeneration.