{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,27]],"date-time":"2026-10-27T21:04:43Z","timestamp":1793135083645,"version":"4.5.0"},"reference-count":164,"publisher":"MDPI AG","issue":"15","license":[{"start":{"date-parts":[[2023,8,2]],"date-time":"2023-08-02T00:00:00Z","timestamp":1690934400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000053","name":"National Eye Institute","doi-asserted-by":"publisher","award":["EY024091"],"award-info":[{"award-number":["EY024091"]}],"id":[{"id":"10.13039\/100000053","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000053","name":"National Eye Institute","doi-asserted-by":"publisher","award":["P30EY019007"],"award-info":[{"award-number":["P30EY019007"]}],"id":[{"id":"10.13039\/100000053","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000053","name":"National Eye Institute","doi-asserted-by":"publisher","award":["EY009076"],"award-info":[{"award-number":["EY009076"]}],"id":[{"id":"10.13039\/100000053","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Department of Ophthalmology, Columbia University","award":["EY024091"],"award-info":[{"award-number":["EY024091"]}]},{"name":"Department of Ophthalmology, Columbia University","award":["P30EY019007"],"award-info":[{"award-number":["P30EY019007"]}]},{"name":"Department of Ophthalmology, Columbia University","award":["EY009076"],"award-info":[{"award-number":["EY009076"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>The method of quantitative fundus autofluorescence (qAF) can be used to assess the levels of bisretinoids in retinal pigment epithelium (RPE) cells so as to aid the interpretation and management of a variety of retinal conditions. In this review, we focused on seven retinal diseases to highlight the possible pathways to increased fundus autofluorescence. ABCA4- and RDH12-associated diseases benefit from known mechanisms whereby gene malfunctioning leads to elevated bisretinoid levels in RPE cells. On the other hand, peripherin2\/RDS-associated disease (PRPH2\/RDS), retinitis pigmentosa (RP), central serous chorioretinopathy (CSC), acute zonal occult outer retinopathy (AZOOR), and ceramide kinase like (CERKL)-associated retinal degeneration all express abnormally high fundus autofluorescence levels without a demonstrated pathophysiological pathway for bisretinoid elevation. We suggest that, while a known link from gene mutation to increased production of bisretinoids (as in ABCA4- and RDH12-associated diseases) causes primary elevation in fundus autofluorescence, a secondary autofluorescence elevation also exists, where an impairment and degeneration of photoreceptor cells by various causes leads to an increase in bisretinoid levels in RPE cells.<\/jats:p>","DOI":"10.3390\/ijms241512327","type":"journal-article","created":{"date-parts":[[2023,8,2]],"date-time":"2023-08-02T11:04:38Z","timestamp":1690974278000},"page":"12327","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Primary versus Secondary Elevations in Fundus Autofluorescence"],"prefix":"10.3390","volume":"24","author":[{"given":"Rait","family":"Parmann","sequence":"first","affiliation":[{"name":"Departments of Ophthalmology, Columbia University, 635 W. 165th Street, New York, NY 10032, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9082-2427","authenticated-orcid":false,"given":"Stephen H.","family":"Tsang","sequence":"additional","affiliation":[{"name":"Departments of Ophthalmology, Columbia University, 635 W. 165th Street, New York, NY 10032, USA"},{"name":"Departments of Pathology and Cell Biology, Columbia University, 635 W. 165th Street, New York, NY 10032, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7448-5950","authenticated-orcid":false,"given":"Janet R.","family":"Sparrow","sequence":"additional","affiliation":[{"name":"Departments of Ophthalmology, Columbia University, 635 W. 165th Street, New York, NY 10032, USA"},{"name":"Departments of Pathology and Cell Biology, Columbia University, 635 W. 165th Street, New York, NY 10032, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2023,8,2]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"7439","DOI":"10.1364\/AO.33.007439","article-title":"Spectrophotometer for noninvasive measurement of intrinsic fluorescence and reflectance of the ocular fundus","volume":"33","author":"Delori","year":"1994","journal-title":"Appl. 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