Article
ER stress in retinal degeneration in S334ter Rho rats.
PloS one - 1 Jan 2012
Shinde Vishal M, Sizova Olga S, Lin Jonathan H, LaVail Matthew M, Gorbatyuk Marina S
Abstract excerpt
The S334ter rhodopsin (Rho) rat (line 4) bears the rhodopsin gene with an early termination codon at residue 334 that is a model for several such mutations found in human patients with autosomal dominant retinitis pigmentosa (ADRP). The Unfolded Protein Response (UPR) is implicated in the pathophysiology of several retinal disorders including ADRP in P23H Rho rats. The aim of this study was to examine the onset...
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