{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T10:04:29Z","timestamp":1790676269550,"version":"4.1.0"},"update-to":[{"DOI":"10.1371\/journal.pgen.1009172","type":"new_version","label":"New version","source":"publisher","updated":{"date-parts":[[2020,11,12]],"date-time":"2020-11-12T00:00:00Z","timestamp":1605139200000}}],"reference-count":54,"publisher":"Public Library of Science (PLoS)","issue":"11","license":[{"start":{"date-parts":[[2020,11,2]],"date-time":"2020-11-02T00:00:00Z","timestamp":1604275200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001809","name":"National Natural Science Foundation of China","doi-asserted-by":"publisher","award":["81870693"],"award-info":[{"award-number":["81870693"]}],"id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["www.plosgenetics.org"],"crossmark-restriction":false},"short-container-title":["PLoS Genet"],"abstract":"<jats:p>\n                    Mutations in the gene rhodopsin are one of the major causes of autosomal dominant retinitis pigmentosa (adRP). Mutant forms of Rhodopsin frequently accumulate in the endoplasmic reticulum (ER), cause ER stress, and trigger photoreceptor cell degeneration. Here, we performed a genome-wide screen to identify suppressors of retinal degeneration in a\n                    <jats:italic>Drosophila<\/jats:italic>\n                    model of adRP, carrying a point mutation in the major rhodopsin, Rh1 (Rh1\n                    <jats:sup>G69D<\/jats:sup>\n                    ). We identified two novel E3 ubiquitin ligases SORDD1 and SORDD2 that effectively suppressed Rh1\n                    <jats:sup>G69D<\/jats:sup>\n                    -induced photoreceptor dysfunction and retinal degeneration. SORDD1\/2 promoted the ubiquitination and degradation of Rh1\n                    <jats:sup>G69D<\/jats:sup>\n                    through VCP (valosin containing protein) and independent of processes reliant on the HRD1 (HMG-CoA reductase degradation protein 1)\/HRD3 complex. We further demonstrate that SORDD1\/2 and HRD1 function in parallel and in a redundant fashion to maintain rhodopsin homeostasis and integrity of photoreceptor cells. These findings identify a new ER-associated protein degradation (ERAD) pathway and suggest that facilitating SORDD1\/2 function may be a therapeutic strategy to treat adRP.\n                  <\/jats:p>","DOI":"10.1371\/journal.pgen.1009172","type":"journal-article","created":{"date-parts":[[2020,11,2]],"date-time":"2020-11-02T13:46:14Z","timestamp":1604324774000},"page":"e1009172","update-policy":"https:\/\/doi.org\/10.1371\/journal.pgen.corrections_policy","source":"Crossref","is-referenced-by-count":14,"title":["Suppression of retinal degeneration by two novel ERAD ubiquitin E3 ligases SORDD1\/2 in Drosophila"],"prefix":"10.1371","volume":"16","author":[{"given":"Jaiwei","family":"Xu","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Haifang","family":"Zhao","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2395-3483","authenticated-orcid":true,"given":"Tao","family":"Wang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"340","published-online":{"date-parts":[[2020,11,2]]},"reference":[{"issue":"7","key":"pgen.1009172.ref001","doi-asserted-by":"crossref","first-page":"3052","DOI":"10.1167\/iovs.05-1443","article-title":"Prevalence of disease-causing mutations in families with autosomal dominant retinitis pigmentosa: a screen of known genes in 200 families","volume":"47","author":"LS Sullivan","year":"2006","journal-title":"Invest Ophthalmol Vis 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