Article
AAV-mediated ERdj5 overexpression protects against P23H rhodopsin toxicity.
Human molecular genetics - 28 May 2020
Aguilà Monica, Bellingham James, Athanasiou Dimitra, Bevilacqua Dalila, Duran Yanai, Maswood Ryea, Parfitt David A, Iwawaki Takao, Spyrou Giannis, Smith Alexander J, Ali Robin R, Cheetham Michael E
Abstract excerpt
Rhodopsin misfolding caused by the P23H mutation is a major cause of autosomal dominant retinitis pigmentosa (adRP). To date, there are no effective treatments for adRP. The BiP co-chaperone and reductase ERdj5 (DNAJC10) is part of the endoplasmic reticulum (ER) quality control machinery, and previous studies have shown that overexpression of ERdj5 in vitro enhanced the degradation of P23H rhodopsin, whereas...
Read the complete abstract on PubMedTopics
Share this publication in a Topic to start or enrich a Post.
