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Using mice with a null mutation in the transcription factor gene Mitf, in which RPE deficiencies are associated with retinal degeneration, we evaluated the role of trophic factors secreted by the RPE in retinal homeostasis. In such mice, the thickness of the outer nuclear layer (ONL) is as in wild type up to postnatal day 10, but then is progressively reduced, associated with a marked increase in the number of apoptotic cells and a decline in staining for rhodopsin. We show that retinal degeneration and decrease in rhodopsin staining can be prevented partially in three different ways: first, by recombining mutant-derived postnatal retina with postnatal wild-type RPE in tissue explant cultures; second, by adding to cultured mutant retina the trophic factor pigment epithelium-derived factor (PEDF; also known as SERPINF1), which is normally produced in RPE under the control of Mitf; and third, by treating the eyes of Mitf mutant mice in vivo with drops containing a bioactive PEDF 17-mer peptide. This latter treatment also led to marked increases in a number of rod and cone genes. The results indicate that RPE-derived trophic factors, in particular PEDF, are instrumental in retinal homeostasis, and suggest that PEDF or its bioactive fragments may have therapeutic potential in RPE deficiency-associated retinal degeneration.<\/jats:p>","DOI":"10.1242\/dmm.035642","type":"journal-article","created":{"date-parts":[[2019,1,11]],"date-time":"2019-01-11T14:06:25Z","timestamp":1547215585000},"source":"Crossref","is-referenced-by-count":39,"title":["Photoreceptor degeneration in microphthalmia (<i>Mitf<\/i>) mice: partial rescue by pigment epithelium-derived factor"],"prefix":"10.1242","volume":"12","author":[{"given":"Yu","family":"Chen","sequence":"first","affiliation":[{"name":"School of Ophthalmology and Optometry and Eye Hospital, State Key Laboratory of Optometry, Ophthalmology, and Vision Science and Zhejiang Provincial Key Laboratory of Ophthalmology, Wenzhou Medical University Laboratory of Developmental Cell Biology and Disease , , Wenzhou 325003 , China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Juan","family":"Yang","sequence":"additional","affiliation":[{"name":"School of Ophthalmology and Optometry and Eye Hospital, State Key Laboratory of Optometry, Ophthalmology, and Vision Science and Zhejiang Provincial Key Laboratory of Ophthalmology, Wenzhou Medical University Laboratory of Developmental Cell Biology and Disease , , Wenzhou 325003 , China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Huiqin","family":"Geng","sequence":"additional","affiliation":[{"name":"School of Ophthalmology and Optometry and Eye Hospital, State Key Laboratory of Optometry, Ophthalmology, and Vision Science and Zhejiang Provincial Key Laboratory of Ophthalmology, Wenzhou Medical University Laboratory of Developmental Cell Biology and Disease , , Wenzhou 325003 , China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Liping","family":"Li","sequence":"additional","affiliation":[{"name":"School of Ophthalmology and Optometry and Eye Hospital, State Key Laboratory of Optometry, Ophthalmology, and Vision Science and Zhejiang Provincial Key Laboratory of Ophthalmology, Wenzhou Medical University Laboratory of Developmental Cell Biology and Disease , , Wenzhou 325003 , China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jinyang","family":"Li","sequence":"additional","affiliation":[{"name":"School of Ophthalmology and Optometry and Eye Hospital, State Key Laboratory of Optometry, Ophthalmology, and Vision Science and Zhejiang Provincial Key Laboratory of Ophthalmology, Wenzhou Medical University Laboratory of Developmental Cell Biology and Disease , , Wenzhou 325003 , China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bing","family":"Cheng","sequence":"additional","affiliation":[{"name":"School of Ophthalmology and Optometry and Eye Hospital, State Key Laboratory of Optometry, Ophthalmology, and Vision Science and Zhejiang Provincial Key Laboratory of Ophthalmology, Wenzhou Medical University Laboratory of Developmental Cell Biology and Disease , , Wenzhou 325003 , China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiaoyin","family":"Ma","sequence":"additional","affiliation":[{"name":"School of Ophthalmology and Optometry and Eye Hospital, State Key Laboratory of Optometry, Ophthalmology, and Vision Science and Zhejiang Provincial Key Laboratory of Ophthalmology, Wenzhou Medical University Laboratory of Developmental Cell Biology and Disease , , Wenzhou 325003 , China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Huirong","family":"Li","sequence":"additional","affiliation":[{"name":"School of Ophthalmology and Optometry and Eye Hospital, State Key Laboratory of Optometry, Ophthalmology, and Vision Science and Zhejiang Provincial Key Laboratory of Ophthalmology, Wenzhou Medical University Laboratory of Developmental Cell Biology and Disease , , Wenzhou 325003 , China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0705-8099","authenticated-orcid":false,"given":"Ling","family":"Hou","sequence":"additional","affiliation":[{"name":"School of Ophthalmology and Optometry and Eye Hospital, State Key Laboratory of Optometry, Ophthalmology, and Vision Science and Zhejiang Provincial Key Laboratory of Ophthalmology, Wenzhou Medical University Laboratory of Developmental Cell Biology and Disease , , Wenzhou 325003 , China"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"237","published-online":{"date-parts":[[2019,1,11]]},"reference":[{"key":"2024061303484066400_DMM035642C1","first-page":"3079","article-title":"Photoreceptor rescue of pigment epithelium-derived factor-impregnated nanoparticles in Royal College of Surgeons rats","volume":"18","author":"Akiyama","year":"2012","journal-title":"Mol. 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