Specific deficits in visual electrophysiology in a mouse model of dominant optic atrophy.
Autosomal dominant optic atrophy (ADOA) is a slowly progressive optic neuropathy caused by mutations in the OPA1 gene. OPA1 is ubiquitously expressed and plays a key role in mitochondrial fusion. Heterozygous Opa1 mutant mice (B6; C3-Opa1(Q285STOP)), have previously been reported to develop visual defects and optic nerve changes. In this study, in vivo visual electrophysiological testing (ERGs and VEPs) was performed on 11-13 month old B6; C3-Opa1(Q285STOP) mice (n = 5) and age/sex matched wi...Expand abstract
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