Mitochondrial Neuropathy

Rotenone is a chemical agent that inhibits mitochondrial electron transport leading to production of reactive oxygen species.  Intravitreal injection of rotenone causes a rapid onset of retinal degeneration. This animal model can be used to test therapeutic intervention on meaningful outcomes including:

• Quantification of retinal ganglion cells in retinal flatmounts
• Quantification of retinal thickness by OCT
• Assessment of retinal function with pattern ERG and full-field ERG
• Assessment of visual acuity with Optokinetic tracking

In addition to general mitochondrial stress, this is a utilized model for Leber’s hereditary optic neuropathy (LHON) which 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.