title: Endoplasmic reticulum stress in vertebrate mutant rhodopsin models of retinal degeneration.
abstract: Rhodopsin mutations cause many types of heritable retinitis pigmentosa (RP). Biochemical and in vitro studies have demonstrated that many RP-linked mutant rhodopsins produce misfolded rhodopsin proteins, which are prone to aggregation and retention within the endoplasmic reticulum, where they cause endoplasmic reticulum stress and activate the Unfolded Protein Response signaling pathways. Many vertebrate models of retinal degeneration have been created through expression of RP-linked rhodopsins in photoreceptors including, but not limited to, VPP/GHL mice, P23H Rhodopsin frogs, P23H rhodopsin rats, S334ter rhodopsin rats, C185R rhodopsin mice, T17M rhodopsin mice, and P23H rhodopsin mice. These models have provided many opportunities to test therapeutic strategies to prevent retinal degeneration and also enabled in vivo investigation of cellular and molecular mechanisms responsible for photoreceptor cell death. Here, we examine and compare the contribution of endoplasmic reticulum stress to retinal degeneration in several vertebrate models of RP generated through expression of mutant rhodopsins.
authors.affiliation: Department of Pathology, University of California, San Diego, USA, hkroeger@ucsd.edu.
authors.forename: Heike
authors.initials: H
authors.lastname: Kroeger
authors.forename: Matthew M
authors.initials: MM
authors.lastname: LaVail
authors.forename: Jonathan H
authors.initials: JH
authors.lastname: Lin
mesh_terms: D000818:Animals; D004195:Disease Models, Animal; D004721:Endoplasmic Reticulum / Q000502:physiology*; D059865:Endoplasmic Reticulum Stress / Q000502:physiology*; D006801:Humans; D051379:Mice; D008822:Mice, Transgenic; D051381:Rats; D055647:Rats, Transgenic; D012162:Retinal Degeneration / Q000235:genetics* / Q000503:physiopathology*; D012243:Rhodopsin / Q000235:genetics*; D013045:Species Specificity; D056811:Unfolded Protein Response / Q000502:physiology; D014714:Vertebrates; D014982:Xenopus laevis
chemical_list: D012243:Rhodopsin