{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,10]],"date-time":"2026-10-10T17:02:20Z","timestamp":1791651740827,"version":"4.3.4"},"reference-count":49,"publisher":"MDPI AG","issue":"21","license":[{"start":{"date-parts":[[2020,10,29]],"date-time":"2020-10-29T00:00:00Z","timestamp":1603929600000},"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":["EY026499, EY10609"],"award-info":[{"award-number":["EY026499, EY10609"]}],"id":[{"id":"10.13039\/100000053","id-type":"DOI","asserted-by":"publisher"}]},{"name":"national institute of helath","award":["GM125528"],"award-info":[{"award-number":["GM125528"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>The large number of inherited retinal disease genes (IRD), including the photopigment rhodopsin and the photoreceptor outer segment (OS) structural component peripherin 2 (PRPH2), has prompted interest in identifying common cellular mechanisms involved in degeneration. Although metabolic dysregulation has been shown to play an important role in the progression of the disease etiology, identifying a common regulator that can preserve the metabolic ecosystem is needed for future development of neuroprotective treatments. Here, we investigated whether retbindin (RTBDN), a rod-specific protein with riboflavin binding capability, and a regulator of riboflavin-derived cofactors flavin mononucleotide (FMN) and flavin adenine dinucleotide (FAD), is protective to the retina in different IRD models; one carrying the P23H mutation in rhodopsin (which causes retinitis pigmentosa) and one carrying the Y141C mutation in Prph2 (which causes a blended cone-rod dystrophy). RTBDN levels are significantly upregulated in both the rhodopsin (Rho)P23H\/+ and Prph2Y141C\/+ retinas. Rod and cone structural and functional degeneration worsened in models lacking RTBDN. In addition, removing Rtbdn worsened other phenotypes, such as fundus flecking. Retinal flavin levels were reduced in RhoP23H\/+\/Rtbdn\u2212\/\u2212 and Prph2Y141C\/+\/Rtbdn\u2212\/\u2212 retinas. Overall, these findings suggest that RTBDN may play a protective role during retinal degenerations that occur at varying rates and due to varying disease mechanisms.<\/jats:p>","DOI":"10.3390\/ijms21218083","type":"journal-article","created":{"date-parts":[[2020,10,29]],"date-time":"2020-10-29T21:21:00Z","timestamp":1604006460000},"page":"8083","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Retbindin: A riboflavin Binding Protein, Is Critical for Photoreceptor Homeostasis and Survival in Models of Retinal Degeneration"],"prefix":"10.3390","volume":"21","author":[{"given":"Ayse M.","family":"Genc","sequence":"first","affiliation":[{"name":"Department of Biomedical Engineering, University of Houston, Houston, TX 77204, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3194-3274","authenticated-orcid":false,"given":"Mustafa S.","family":"Makia","sequence":"additional","affiliation":[{"name":"Department of Biomedical Engineering, University of Houston, Houston, TX 77204, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7138-0907","authenticated-orcid":false,"given":"Tirthankar","family":"Sinha","sequence":"additional","affiliation":[{"name":"Department of Biomedical Engineering, University of Houston, Houston, TX 77204, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shannon M.","family":"Conley","sequence":"additional","affiliation":[{"name":"Department of Cell Biology, University of Oklahoma Health Sciences Center, Oklahoma City, OK 73104, USA"},{"name":"Oklahoma Center for Neurosciences, University of Oklahoma Health Sciences Center, Oklahoma City, OK 73104, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Muayyad R.","family":"Al-Ubaidi","sequence":"additional","affiliation":[{"name":"Department of Biomedical Engineering, University of Houston, Houston, TX 77204, USA"},{"name":"College of Optometry, University of Houston, Houston, TX 77204, USA"},{"name":"Department of Biology and Biochemistry, University of Houston, TX 77204, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Muna I.","family":"Naash","sequence":"additional","affiliation":[{"name":"Department of Biomedical Engineering, University of Houston, Houston, TX 77204, USA"},{"name":"College of Optometry, University of Houston, Houston, TX 77204, USA"},{"name":"Department of Biology and Biochemistry, University of Houston, TX 77204, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2020,10,29]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"5041","DOI":"10.1074\/jbc.M114.624189","article-title":"Retbindin is an extracellular riboflavin-binding protein found at the photoreceptor\/retinal pigment epithelium interface","volume":"290","author":"Kelley","year":"2015","journal-title":"J. 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