Endoplasmic reticulum (ER) Ca²⁺-channel activity contributes to ER stress and cone death in cyclic nucleotide-gated channel deficiency.

ABSTRACT: Endoplasmic reticulum (ER) stress and mislocalization of improperly folded proteins have been shown to contribute to photoreceptor death in models of inherited retinal degenerative diseases. In particular, mice with cone cyclic nucleotide-gated (CNG) channel deficiency, a model for achromatopsia, display both early-onset ER stress and opsin mistrafficking. By 2 weeks of age, these mice show elevated signaling from all three arms of the ER-stress pathway, and by 1 month, cone opsin is improperly distributed away from its normal outer segment location to other retinal layers. This work investigated the role of Ca²⁺-release channels in ER stress, protein mislocalization, and cone death in a mouse model of CNG-channel deficiency. We examined whether preservation of luminal Ca²⁺

SUBMITTER: Butler MR 

PROVIDER: S-EPMC5500788 | biostudies-literature | 2017 Jul

REPOSITORIES: biostudies-literature