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The P23H mutation in rhodopsin (Rho P23H ) is a prevalent cause of autosomal dominant retinitis pigmentosa. We examined the role of the ER stress proteins, Chop and Ask1, in regulating the death of rod photoreceptors in a mouse line harboring the Rho P23H  rhodopsin transgene ( GHL +  ). We used knockout mice models to determine whether Chop and Ask1 regulate rod survival or retinal degeneration. Electrophysiological recordings showed similar retinal responses and sensitivities for  GHL +  ,  GHL + /Chop −/−   and  GHL + /Ask1 −/−   animals between 4–28 weeks, by which time all three mouse lines exhibited severe loss of retinal function. Histologically, ablation of  Chop  and  Ask1  did not rescue photoreceptor loss in young animals. However, in older mice, a regional protective effect was observed in the central retina of  GHL + /Chop −/−   and  GHL + /Ask1 −/−  , a region that was severely degenerated in  GHL +   mice. Our results show that in the presence of the Rho P23H  transgene, the rate of decline in retinal sensitivity is similar in  Chop  or  Ask1  ablated and wild-type retinas, suggesting that these proteins do not play a major role during the acute phase of photoreceptor loss in  GHL +   mice. Instead they may be involved in regulating secondary pathological responses such as inflammation that are upregulated during later stages of disease progression.

plos one

Ablation of the Proapoptotic Genes Chop or Ask1 Does Not Prevent or Delay Loss of Visual Function in a P23H Transgenic Mouse Model of Retinitis Pigmentosa