A dual role for EDEM1 in the processing of rod opsin.

Journal of Cell Science
Maria KosmaoglouMichael E Cheetham

Abstract

Mutations in rod opsin, the archetypal G-protein-coupled receptor, cause retinitis pigmentosa. The majority of mutations, e.g. P23H, cause protein misfolding, resulting in ER retention, induction of the unfolded protein response and degradation by ERAD. If misfolded rod opsin escapes degradation, it aggregates and forms intracellular inclusions. Therefore, it is important to identify the chaperones that mediate the folding or degradation of rod opsin. ER degradation enhancing alpha-mannosidase-like 1 (EDEM1) can enhance the release of terminally misfolded glycoproteins from the calnexin chaperone system. Here, we identify EDEM1 as a novel chaperone of rod opsin. EDEM1 expression promoted the degradation of P23H rod opsin and decreased its aggregation. By contrast, shRNA-mediated knockdown of EDEM1 increased both the amount of P23H rod opsin and its aggregation into inclusions. EDEM1 was detected in rod photoreceptor inner segments and EndoH-sensitive rod opsin co-immunoprecipitated with EDEM1 from retina, suggesting that rod opsin is a physiological EDEM1 client. Unexpectedly, EDEM1 binding to rod opsin was independent of mannose trimming and EDEM1 promoted the cell-surface expression of mutant rod opsin. Collectively, the data...Continue Reading

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Citations

Sep 24, 2011·The Journal of Biological Chemistry·Darwin ToledoPere Garriga
Jun 3, 2011·The Journal of Biological Chemistry·Taku TamuraDaniel N Hebert
Jan 6, 2012·Molecular Biology of the Cell·Wei-Chieh ChiangJonathan H Lin
Aug 3, 2012·Molecular Biology of the Cell·Dimitra AthanasiouMichael E Cheetham
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Sep 30, 2020·Genes·Fay Newton, Roly Megaw
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