Yang, H., Y. Fu, X. Liu, P. Shahi, T. Mavlyutov, J. Li, A. Yao, S. Guo, B. Pattnaik, and L. Guo. Role of the Sigma-1 Receptor Chaperone in Rod and Cone Photoreceptor Degenerations in a Mouse Model of Retinitis Pigmentosa. Mol Neurodegener, 2017, p. PubMed Text.
Abstract
Background: Retinitis pigmentosa (RP) is the most common inherited retinal degenerative disease yet with no effective treatment available. The sigma-1 receptor (S1R), a ligand-regulated chaperone, emerges as a potential retina-protective therapeutic target. In particular, pharmacological activation of S1R was recently shown to rescue cones in the rd10 mouse, a rod Pde6b mutant that recapitulates the RP pathology of autonomous rod degeneration followed by secondary death of cones. The mechanisms underlying the S1R protection for cones are not understood in detail.
Keywords: The sigma-1 receptor; autophagy; electroretinography (ERG); necroptosis; rd10/S1R−/− and rd10/S1R+/+ mice.