We conducted a trial of idebenone in an Opa1 mouse model of dominant optic atrophy.
We report the expression profile of NQO1 in the brain, retina and liver.
Mutant mice receiving treatment showed an early improvement in visual acuity.
In aged mice, vision and retinal dendritic loss were not prevented by idebenone.
Idebenone had a deleterious effect on wildtype retinal ganglion cells.