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RPGR plays a critical role as a scaffold protein in the regulation of protein trafficking from the basal body to the axoneme, where the cargoes are transported to the outer segments (OSs) of photoreceptors. This trafficking process is controlled directly by intraflagellar transport complexes and regulated by the RPGR protein complex, although the precise mechanisms have yet to be defined. We used an\n                    <jats:italic>Rpgr<\/jats:italic>\n                    conditional knockout (cko) mouse model to investigate the disease mechanisms during retinal degeneration and to evaluate the protective effects of tauroursodeoxycholic acid (TUDCA). Rhodopsin, cone opsins and transducin were mislocalized in\n                    <jats:italic>Rpgr<\/jats:italic>\n                    cko photoreceptors, while localization of NPHP4 to connecting cilia was absent, suggesting that RPGR is required for ciliary protein trafficking. Microglia were activated in advance of retinal degeneration in\n                    <jats:italic>Rpgr<\/jats:italic>\n                    cko mouse retinas. TUDCA treatment suppressed microglial activation and inflammation and prevented photoreceptor degeneration in\n                    <jats:italic>Rpgr<\/jats:italic>\n                    cko mice. Our data demonstrated that TUDCA has therapeutic potential for RPGR\u2010associated RP patients.\n                  <\/jats:p>","DOI":"10.1002\/jcp.28519","type":"journal-article","created":{"date-parts":[[2019,3,29]],"date-time":"2019-03-29T00:06:31Z","timestamp":1553817991000},"page":"18801-18812","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":29,"title":["Disease mechanisms and neuroprotection by tauroursodeoxycholic acid in\n                    <i>Rpgr<\/i>\n                    knockout mice"],"prefix":"10.1002","volume":"234","author":[{"given":"Xun","family":"Zhang","sequence":"first","affiliation":[{"name":"Department of Biological and Biomedical Sciences Glasgow Caledonian University Glasgow Scotland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Uma","family":"Shahani","sequence":"additional","affiliation":[{"name":"Department of Vision Science Glasgow Caledonian University Glasgow Scotland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James","family":"Reilly","sequence":"additional","affiliation":[{"name":"Department of Biological and Biomedical Sciences Glasgow Caledonian University Glasgow Scotland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3760-3019","authenticated-orcid":false,"given":"Xinhua","family":"Shu","sequence":"additional","affiliation":[{"name":"Department of Biological and Biomedical Sciences Glasgow Caledonian University Glasgow Scotland"},{"name":"Department of Vision Science Glasgow Caledonian University Glasgow Scotland"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2019,3,28]]},"reference":[{"key":"e_1_2_8_1_2_1","doi-asserted-by":"publisher","DOI":"10.1167\/iovs.05-0845"},{"key":"e_1_2_8_1_3_1","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2017.01930"},{"key":"e_1_2_8_1_4_1","first-page":"1706","article-title":"Tool from ancient pharmacopeia prevents vision loss","volume":"12","author":"Boatright J. 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