{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,1]],"date-time":"2026-09-01T12:26:58Z","timestamp":1788265618825,"version":"build-2803163510"},"reference-count":89,"publisher":"Elsevier BV","issue":"3","license":[{"start":{"date-parts":[[2026,3,1]],"date-time":"2026-03-01T00:00:00Z","timestamp":1772323200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2026,3,1]],"date-time":"2026-03-01T00:00:00Z","timestamp":1772323200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2026,2,4]],"date-time":"2026-02-04T00:00:00Z","timestamp":1770163200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100001116","name":"Foundation Fighting Blindness","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100001116","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000053","name":"National Eye Institute","doi-asserted-by":"publisher","award":["RO1EY012951"],"award-info":[{"award-number":["RO1EY012951"]}],"id":[{"id":"10.13039\/100000053","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100002509","name":"Keimyung University","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100002509","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["jbc.org","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Journal of Biological Chemistry"],"published-print":{"date-parts":[[2026,3]]},"DOI":"10.1016\/j.jbc.2026.111261","type":"journal-article","created":{"date-parts":[[2026,2,5]],"date-time":"2026-02-05T16:00:44Z","timestamp":1770307244000},"page":"111261","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":4,"title":["More insights from Abca4 mouse models of recessive Stargardt disease"],"prefix":"10.1016","volume":"302","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1934-6445","authenticated-orcid":false,"given":"Jin","family":"Zhao","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Diego","family":"Montenegro","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4632-7362","authenticated-orcid":false,"given":"Sihua","family":"Cheng","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hye Jin","family":"Kim","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7448-5950","authenticated-orcid":false,"given":"Janet R.","family":"Sparrow","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.jbc.2026.111261_bib1","doi-asserted-by":"crossref","first-page":"523","DOI":"10.1023\/A:1012883306823","article-title":"Mechanistic studies of ABCR, the ABC transporter in photoreceptor outer segments responsible for autosomal recessive Stargardt disease","volume":"33","author":"Sun","year":"2001","journal-title":"J. Bioenerg. Biomembrane"},{"key":"10.1016\/j.jbc.2026.111261_bib2","doi-asserted-by":"crossref","first-page":"242","DOI":"10.1038\/79994","article-title":"Biochemical defects in ABCR protein variants associated with human retinopathies","volume":"26","author":"Sun","year":"2000","journal-title":"Nat. Genet."},{"key":"10.1016\/j.jbc.2026.111261_bib3","doi-asserted-by":"crossref","first-page":"8269","DOI":"10.1074\/jbc.274.12.8269","article-title":"Retinal stimulates ATP hydrolysis by purified and reconstituted ABCR, the photoreceptor-specific ATP-binding cassette transporter responsible for Stargardt disease","volume":"274","author":"Sun","year":"1999","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.jbc.2026.111261_bib4","doi-asserted-by":"crossref","first-page":"257","DOI":"10.1038\/77004","article-title":"ABCR expression in foveal cone photoreceptors and its role in Stargardt macular dystrophy","volume":"25","author":"Molday","year":"2000","journal-title":"Nat. Genet."},{"key":"10.1016\/j.jbc.2026.111261_bib5","doi-asserted-by":"crossref","first-page":"E11120","DOI":"10.1073\/pnas.1802519115","article-title":"Expression of ABCA4 in the retinal pigment epithelium and its implications for Stargardt macular degeneration","volume":"115","author":"Lenis","year":"2018","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.jbc.2026.111261_bib6","doi-asserted-by":"crossref","first-page":"14609","DOI":"10.1073\/pnas.95.25.14609","article-title":"Isolation and one-step preparation of A2E and iso-A2E, fluorophores from human retinal pigment epithelium","volume":"95","author":"Parish","year":"1998","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.jbc.2026.111261_bib7","doi-asserted-by":"crossref","first-page":"5024","DOI":"10.1073\/pnas.1400780111","article-title":"ATP-binding cassette transporter ABCA4 and chemical isomerization protect photoreceptor cells from the toxic accumulation of excess 11-cis-retinal","volume":"111","author":"Quazi","year":"2014","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.jbc.2026.111261_bib8","doi-asserted-by":"crossref","DOI":"10.1016\/j.preteyeres.2021.101036","article-title":"Structure and function of ABCA4 and its role in the visual cycle and Stargardt macular degeneration","volume":"89","author":"Molday","year":"2022","journal-title":"Prog. Retin. Eye Res."},{"key":"10.1016\/j.jbc.2026.111261_bib9","doi-asserted-by":"crossref","first-page":"1559","DOI":"10.1021\/ja953480g","article-title":"Ocular age pigment \"A2E\": an unprecedented pyridinium bisretinoid","volume":"118","author":"Sakai","year":"1996","journal-title":"J. Am. Chem. Soc."},{"key":"10.1016\/j.jbc.2026.111261_bib10","doi-asserted-by":"crossref","first-page":"3619","DOI":"10.1021\/ja9700414","article-title":"Total synthesis of the ocular age pigment A2E: a convergent pathway","volume":"119","author":"Ren","year":"1997","journal-title":"J. Am. Chem. Soc."},{"key":"10.1016\/j.jbc.2026.111261_bib11","doi-asserted-by":"crossref","first-page":"19273","DOI":"10.1073\/pnas.0708714104","article-title":"The all-trans-retinal dimer series of lipofuscin pigments in retinal pigment epithelial cells in a recessive Stargardt disease model","volume":"104","author":"Kim","year":"2007","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.jbc.2026.111261_bib12","doi-asserted-by":"crossref","first-page":"20155","DOI":"10.1074\/jbc.M109.021345","article-title":"Novel lipofuscin bisretinoids prominent in human retina and in a model of recessive Stargardt disease","volume":"284","author":"Wu","year":"2009","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.jbc.2026.111261_bib13","doi-asserted-by":"crossref","first-page":"1620","DOI":"10.1194\/jlr.M084459","article-title":"Novel bisretinoids of human retina are lyso alkyl ether glycerophosphoethanolamine-bearing A2PE species","volume":"59","author":"Kim","year":"2018","journal-title":"J. Lipid Res."},{"key":"10.1016\/j.jbc.2026.111261_bib14","doi-asserted-by":"crossref","DOI":"10.1016\/j.preteyeres.2025.101388","article-title":"Bisretinoid lipofuscin, fundus autofluorescence and retinal disease","volume":"108","author":"Sparrow","year":"2025","journal-title":"Prog. Retin. Eye Res."},{"key":"10.1016\/j.jbc.2026.111261_bib15","doi-asserted-by":"crossref","first-page":"392","DOI":"10.1083\/jcb.42.2.392","article-title":"Participation of the retinal pigment epithelium in the rod outer segment renewal process","volume":"42","author":"Young","year":"1969","journal-title":"J. Cell Biol."},{"key":"10.1016\/j.jbc.2026.111261_bib16","first-page":"495","article-title":"Stargardt macular dystrophy and therapeutic approaches","volume":"108","author":"Fujinami","year":"2024","journal-title":"Br. J. Ophthalmol."},{"key":"10.1016\/j.jbc.2026.111261_bib17","doi-asserted-by":"crossref","first-page":"165","DOI":"10.3109\/13816810.2011.565397","article-title":"Allelic and phenotypic heterogeneity in ABCA4 mutations","volume":"32","author":"Burke","year":"2011","journal-title":"Ophthalmic Genet."},{"key":"10.1016\/j.jbc.2026.111261_bib18","doi-asserted-by":"crossref","first-page":"504","DOI":"10.1001\/archopht.117.4.504","article-title":"Variation of clinical expression in patients with Stargardt dystrophy and sequence variations in the ABCR gene","volume":"117","author":"Fishman","year":"1999","journal-title":"Arch. Ophthalmol."},{"key":"10.1016\/j.jbc.2026.111261_bib19","doi-asserted-by":"crossref","first-page":"359","DOI":"10.1001\/archopht.119.3.359","article-title":"Phenotypic subtypes of Stargardt macular dystrophy-fundus flavimaculatus","volume":"119","author":"Lois","year":"2001","journal-title":"Arch. Ophthalmol."},{"key":"10.1016\/j.jbc.2026.111261_bib20","doi-asserted-by":"crossref","first-page":"159","DOI":"10.1016\/j.ajo.2019.11.008","article-title":"Prospective cohort study of childhood-onset Stargardt disease: fundus autofluorescence imaging, progression, comparison with adult-onset disease, and disease symmetry","volume":"211","author":"Georgiou","year":"2020","journal-title":"Am. J. Ophthalmol."},{"key":"10.1016\/j.jbc.2026.111261_bib21","doi-asserted-by":"crossref","first-page":"1199","DOI":"10.1016\/j.ophtha.2012.01.005","article-title":"Clinical and genetic characteristics of late-onset Stargardt's disease","volume":"119","author":"Westeneng-van Haaften","year":"2012","journal-title":"Ophthalmology"},{"key":"10.1016\/j.jbc.2026.111261_bib22","doi-asserted-by":"crossref","first-page":"11","DOI":"10.1186\/1471-2350-15-11","article-title":"Whole exome sequencing detects homozygosity for ABCA4 p.Arg602Trp missense mutation in a pediatric patient with rapidly progressive retinal dystrophy","volume":"15","author":"Ortube","year":"2014","journal-title":"BMC Med. Genet."},{"key":"10.1016\/j.jbc.2026.111261_bib23","doi-asserted-by":"crossref","first-page":"5946","DOI":"10.1167\/iovs.15-17698","article-title":"Predicting progression of ABCA4-Associated retinal degenerations based on longitudinal measurements of the leading disease front","volume":"56","author":"Cideciyan","year":"2015","journal-title":"Invest Ophthalmol. Vis. Sci."},{"key":"10.1016\/j.jbc.2026.111261_bib24","doi-asserted-by":"crossref","first-page":"525","DOI":"10.1093\/hmg\/ddh048","article-title":"Mutations in ABCA4 result in accumulation of lipofuscin before slowing of the retinoid cycle: a reappraisal of the human disease sequence","volume":"13","author":"Cideciyan","year":"2004","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.jbc.2026.111261_bib25","series-title":"Origin of Fundus Autofluorescence in Atlas of Fundus Autofluorescence Imaging","first-page":"17","author":"Delori","year":"2007"},{"key":"10.1016\/j.jbc.2026.111261_bib26","first-page":"2327","article-title":"In vivo measurement of lipofuscin in Stargardt's disease--Fundus flavimaculatus","volume":"36","author":"Delori","year":"1995","journal-title":"Invest Ophthalmol. Vis. Sci."},{"key":"10.1016\/j.jbc.2026.111261_bib27","doi-asserted-by":"crossref","first-page":"2841","DOI":"10.1167\/iovs.13-13624","article-title":"Quantitative fundus autofluorescence in recessive Stargardt disease","volume":"55","author":"Burke","year":"2014","journal-title":"Invest Ophthalmol. Vis. Sci."},{"key":"10.1016\/j.jbc.2026.111261_bib28","doi-asserted-by":"crossref","first-page":"13","DOI":"10.1016\/S0092-8674(00)80602-9","article-title":"Insights into the function of Rim protein in photoreceptors and etiology of Stargardt's disease from the phenotype in abcr knockout mice","volume":"98","author":"Weng","year":"1999","journal-title":"Cell"},{"key":"10.1016\/j.jbc.2026.111261_bib29","doi-asserted-by":"crossref","first-page":"11668","DOI":"10.1073\/pnas.0403499101","article-title":"The Rpe65 Leu450Met variant is associated with reduced levels of the RPE lipofuscin fluorophores A2E and iso-A2E","volume":"101","author":"Kim","year":"2004","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.jbc.2026.111261_bib30","doi-asserted-by":"crossref","first-page":"5435","DOI":"10.1167\/iovs.09-3944","article-title":"Limited roles of Rdh8, Rdh12, and Abca4 in all-trans-retinal clearance in mouse retina","volume":"50","author":"Maeda","year":"2009","journal-title":"Invest Ophthalmol. Vis. Sci."},{"key":"10.1016\/j.jbc.2026.111261_bib31","doi-asserted-by":"crossref","first-page":"533","DOI":"10.1007\/978-1-4419-1399-9_61","article-title":"Photoreceptor cell degeneration in Abcr-\/- mice","volume":"664","author":"Wu","year":"2010","journal-title":"Adv. Exp. Med. Biol."},{"key":"10.1016\/j.jbc.2026.111261_bib32","doi-asserted-by":"crossref","first-page":"1910","DOI":"10.1167\/iovs.14-13867","article-title":"Light damage in Abca4 and Rpe65rd12 mice","volume":"55","author":"Wu","year":"2014","journal-title":"Invest Ophthalmol. Vis. Sci."},{"key":"10.1016\/j.jbc.2026.111261_bib33","doi-asserted-by":"crossref","first-page":"3220","DOI":"10.1093\/hmg\/ddv073","article-title":"Protein misfolding and the pathogenesis of ABCA4-associated retinal degenerations","volume":"24","author":"Zhang","year":"2015","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.jbc.2026.111261_bib34","doi-asserted-by":"crossref","first-page":"26684","DOI":"10.1074\/jbc.M804505200","article-title":"Retinopathy in mice induced by disrupted all-trans-retinal clearance","volume":"283","author":"Maeda","year":"2008","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.jbc.2026.111261_bib35","series-title":"Adler\u2019s Physiology of the Eye","article-title":"Electroretinogram of Human, Monkey And Mouse","author":"Frishman","year":"2011"},{"key":"10.1016\/j.jbc.2026.111261_bib36","doi-asserted-by":"crossref","first-page":"295","DOI":"10.1007\/978-1-60327-325-1_17","article-title":"Diet in vitamin A research","volume":"652","author":"Ross","year":"2010","journal-title":"Methods Mol. Biol."},{"key":"10.1016\/j.jbc.2026.111261_bib37","doi-asserted-by":"crossref","first-page":"6767","DOI":"10.1074\/jbc.RA120.012695","article-title":"Effects of deficiency in the RLBP1-encoded visual cycle protein CRALBP on visual dysfunction in humans and mice","volume":"295","author":"Lima de Carvalho","year":"2020","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.jbc.2026.111261_bib38","doi-asserted-by":"crossref","DOI":"10.1194\/jlr.TR120000742","article-title":"Bisretinoid phospholipid and vitamin A aldehyde: shining a light","volume":"62","author":"Kim","year":"2021","journal-title":"J. Lipid Res."},{"key":"10.1016\/j.jbc.2026.111261_bib39","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1016\/0163-7827(83)90004-8","article-title":"Chemistry and metabolism of lipids inthe vertebrate retina","volume":"22","author":"Fliesler","year":"1983","journal-title":"Prog. Lipid Res."},{"key":"10.1016\/j.jbc.2026.111261_bib40","doi-asserted-by":"crossref","first-page":"353","DOI":"10.1016\/S0962-8924(98)01341-5","article-title":"Vertebrate photoreceptor cell development and disease","volume":"8","author":"Morrow","year":"1998","journal-title":"Trends Cell Biol"},{"key":"10.1016\/j.jbc.2026.111261_bib41","doi-asserted-by":"crossref","first-page":"8936","DOI":"10.1523\/JNEUROSCI.18-21-08936.1998","article-title":"The major cell populations of the mouse retina","volume":"18","author":"Jeon","year":"1998","journal-title":"J. Neurosci."},{"key":"10.1016\/j.jbc.2026.111261_bib42","doi-asserted-by":"crossref","first-page":"2812","DOI":"10.1167\/iovs.12-11490","article-title":"Quantitative fundus autofluorescence in mice: correlation with HPLC quantitation of RPE lipofuscin and measurement of retina outer nuclear layer thickness","volume":"54","author":"Sparrow","year":"2013","journal-title":"Invest Ophthalmol. Vis. Sci."},{"key":"10.1016\/j.jbc.2026.111261_bib43","doi-asserted-by":"crossref","first-page":"2459","DOI":"10.1167\/iovs.18-24213","article-title":"Mutations in GPR143\/OA1 and ABCA4 inform interpretations of short-wavelength and near-infrared fundus autofluorescence","volume":"59","author":"Paavo","year":"2018","journal-title":"Invest Ophthalmol. Vis. Sci."},{"key":"10.1016\/j.jbc.2026.111261_bib44","doi-asserted-by":"crossref","first-page":"3329","DOI":"10.3390\/cells11203329","article-title":"Assessment of choroidal vasculature and innate immune cells in the eyes of albino and pigmented mice","volume":"11","author":"Zaitoun","year":"2022","journal-title":"Cells"},{"key":"10.1016\/j.jbc.2026.111261_bib45","doi-asserted-by":"crossref","first-page":"6904","DOI":"10.1073\/pnas.1524774113","article-title":"Photodegradation of retinal bisretinoids in mouse models and implications for macular degeneration","volume":"113","author":"Ueda","year":"2016","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.jbc.2026.111261_bib46","doi-asserted-by":"crossref","first-page":"22276","DOI":"10.1074\/jbc.M111.329235","article-title":"Lipofuscin and N-retinylidene-N-retinylethanolamine (A2E) accumulate in the retinal pigment epithelium in the absence of light exposure: their origin is 11-cis retinal","volume":"287","author":"Boyer","year":"2012","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.jbc.2026.111261_bib47","article-title":"Photobleaching and fluorescence recovery of RPE bisretinoids","volume":"10","author":"Liu","year":"2015","journal-title":"PLoS One"},{"key":"10.1016\/j.jbc.2026.111261_bib48","doi-asserted-by":"crossref","first-page":"27215","DOI":"10.1074\/jbc.M115.680363","article-title":"Correlations between photodegradation of bisretinoid constituents of retina and dicarbonyl-adduct deposition","volume":"290","author":"Zhou","year":"2015","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.jbc.2026.111261_bib49","first-page":"1839","article-title":"Photo-products of retinal pigment epithelial bisretinoids react with cellular thiols","volume":"17","author":"Yoon","year":"2011","journal-title":"Mol. Vis."},{"key":"10.1016\/j.jbc.2026.111261_bib50","doi-asserted-by":"crossref","first-page":"3536","DOI":"10.1167\/iovs.12-9535","article-title":"Toward an understanding of bisretinoid autofluorescence bleaching and recovery","volume":"53","author":"Yamamoto","year":"2012","journal-title":"Invest Ophthalmol. Vis. Sci."},{"key":"10.1016\/j.jbc.2026.111261_bib51","doi-asserted-by":"crossref","first-page":"19565","DOI":"10.1073\/pnas.0707477104","article-title":"Redundant and unique roles of retinol dehydrogenases in the mouse retina","volume":"104","author":"Maeda","year":"2007","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.jbc.2026.111261_bib52","doi-asserted-by":"crossref","first-page":"256","DOI":"10.1089\/rej.2006.9.256","article-title":"Indirect antioxidant protection against photooxidative processes initiated in retinal pigment epithelial cells by a lipofuscin pigment","volume":"9","author":"Zhou","year":"2006","journal-title":"Rejuven Res."},{"key":"10.1016\/j.jbc.2026.111261_bib53","doi-asserted-by":"crossref","first-page":"529","DOI":"10.1111\/j.1751-1097.2005.tb00221.x","article-title":"Anthocyanins protect against A2E photooxidation and membrane permeabilization in retinal pigment epithelial cells","volume":"81","author":"Jang","year":"2005","journal-title":"Photochem. Photobiol."},{"key":"10.1016\/j.jbc.2026.111261_bib54","doi-asserted-by":"crossref","first-page":"18207","DOI":"10.1074\/jbc.M300457200","article-title":"A2E-epoxides damage DNA in retinal pigment epithelial cells. Vitamin E and other antioxidants inhibit A2E-epoxide formation","volume":"278","author":"Sparrow","year":"2003","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.jbc.2026.111261_bib55","doi-asserted-by":"crossref","first-page":"964","DOI":"10.1016\/j.ajo.2015.02.004","article-title":"The effect of light deprivation in patients with Stargardt disease","volume":"159","author":"Teussink","year":"2015","journal-title":"Am. J. Ophthalmol."},{"key":"10.1016\/j.jbc.2026.111261_bib56","doi-asserted-by":"crossref","first-page":"4353","DOI":"10.1073\/pnas.1031478100","article-title":"Therapy for macular degeneration: insights from acne","volume":"100","author":"Sparrow","year":"2003","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.jbc.2026.111261_bib57","doi-asserted-by":"crossref","first-page":"3821","DOI":"10.1167\/iovs.07-1470","article-title":"Accelerated accumulation of lipofuscin pigments in the RPE of a mouse model for ABCA4-mediated retinal dystrophies following vitamin A supplementation","volume":"49","author":"Radu","year":"2008","journal-title":"Invest Ophthalmol. Vis. Sci."},{"key":"10.1016\/j.jbc.2026.111261_bib58","doi-asserted-by":"crossref","DOI":"10.1016\/j.jbc.2023.104784","article-title":"A high fat diet fosters elevated bisretinoids","volume":"299","author":"Kim","year":"2023","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.jbc.2026.111261_bib89","doi-asserted-by":"crossref","first-page":"573","DOI":"10.1016\/j.bbalip.2009.02.004","article-title":"The role of the photoreceptor ABC transporter ABCA4 in lipid transport and Stargardt macular degeneration","volume":"1791","author":"Molday","year":"2009","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.jbc.2026.111261_bib59","doi-asserted-by":"crossref","first-page":"3131","DOI":"10.1007\/s00417-022-05684-9","article-title":"Intrinsic differences in rod and cone membrane composition: implications for cone degeneration","volume":"260","author":"Verra","year":"2022","journal-title":"Graefes Arch. Clin. Exp. Ophthalmol."},{"key":"10.1016\/j.jbc.2026.111261_bib60","doi-asserted-by":"crossref","first-page":"1169","DOI":"10.1016\/j.bbadis.2011.10.007","article-title":"Increased cone sensitivity to ABCA4 deficiency provides insight into macular vision loss in Stargardt's dystrophy","volume":"1822","author":"Conley","year":"2012","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.jbc.2026.111261_bib61","doi-asserted-by":"crossref","first-page":"24867","DOI":"10.1073\/pnas.2005714117","article-title":"Vitamin A aldehyde-taurine adduct and the visual cycle","volume":"117","author":"Kim","year":"2020","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.jbc.2026.111261_bib62","doi-asserted-by":"crossref","first-page":"121","DOI":"10.1016\/j.preteyeres.2011.12.001","article-title":"The bisretinoids of retinal pigment epithelium","volume":"31","author":"Sparrow","year":"2012","journal-title":"Prog. Retin. Eye Res."},{"key":"10.1016\/j.jbc.2026.111261_bib63","doi-asserted-by":"crossref","DOI":"10.1016\/j.jbc.2024.105781","article-title":"Unraveling the mystery of ocular retinoid turnover: insights from albino mice and the role of STRA6","volume":"300","author":"Ramkumar","year":"2024","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.jbc.2026.111261_bib64","doi-asserted-by":"crossref","first-page":"17551","DOI":"10.1073\/pnas.1008769107","article-title":"Inhibition of the visual cycle by A2E through direct interaction with RPE65 and implications in Stargardt disease","volume":"107","author":"Moiseyev","year":"2010","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.jbc.2026.111261_bib65","doi-asserted-by":"crossref","first-page":"5029","DOI":"10.1167\/iovs.15-16763","article-title":"Flecks in recessive Stargardt disease: short-wavelength autofluorescence, near-infrared autofluorescence, and optical coherence tomographyDisease","volume":"56","author":"Sparrow","year":"2015","journal-title":"Invest Ophthalmol. Vis. Sci."},{"key":"10.1016\/j.jbc.2026.111261_bib66","doi-asserted-by":"crossref","first-page":"98","DOI":"10.1002\/jnr.24252","article-title":"Photoreceptor cells as a source of fundus autofluorescence in recessive Stargardt disease","volume":"97","author":"Paavo","year":"2019","journal-title":"J. Neurosci. Res."},{"key":"10.1016\/j.jbc.2026.111261_bib67","doi-asserted-by":"crossref","first-page":"5227","DOI":"10.1167\/iovs.17-22532","article-title":"A Comparison of En Face Optical Coherence Tomography and Fundus Autofluorescence in Stargardt Disease","volume":"58","author":"Greenstein","year":"2017","journal-title":"Invest Ophthalmol. Vis. Sci."},{"key":"10.1016\/j.jbc.2026.111261_bib68","doi-asserted-by":"crossref","first-page":"274","DOI":"10.1167\/iovs.18-25755","article-title":"Spectrally resolved fundus autofluorescence in ABCA4-Related retinopathy","volume":"60","author":"Dysli","year":"2019","journal-title":"Invest Ophthalmol. Vis. Sci."},{"key":"10.1016\/j.jbc.2026.111261_bib69","doi-asserted-by":"crossref","first-page":"1211","DOI":"10.1016\/S0161-6420(13)31725-4","article-title":"Histopathology and immunocytochemistry of the neurosensory retina in fundus flavimaculatus","volume":"101","author":"Birnbach","year":"1994","journal-title":"Ophthalmology"},{"key":"10.1016\/j.jbc.2026.111261_bib70","doi-asserted-by":"crossref","DOI":"10.3389\/fopht.2023.1305864","article-title":"Iron overload and chelation modulates bisretinoid levels in the retina","volume":"3","author":"Zhao","year":"2023","journal-title":"Front. Ophthalmol."},{"key":"10.1016\/j.jbc.2026.111261_bib71","doi-asserted-by":"crossref","DOI":"10.1016\/j.jbc.2022.102722","article-title":"Products of the visual cycle are detected in mice lacking retinol binding protein 4, the only known vitamin A carrier in plasma","volume":"298","author":"Montenegro","year":"2022","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.jbc.2026.111261_bib72","doi-asserted-by":"crossref","DOI":"10.1073\/pnas.2314698120","article-title":"Distinct mouse models of Stargardt disease display differences in pharmacological targeting of ceramides and inflammatory responses","volume":"120","author":"Engfer","year":"2023","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.jbc.2026.111261_bib73","doi-asserted-by":"crossref","first-page":"5551","DOI":"10.1073\/pnas.97.10.5551","article-title":"A deletion in a photoreceptor-specific nuclear receptor mRNA causes retinal degeneration in the rd7 mouse","volume":"97","author":"Akhmedov","year":"2000","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.jbc.2026.111261_bib74","doi-asserted-by":"crossref","first-page":"192","DOI":"10.1016\/j.exer.2015.04.009","article-title":"The BALB\/c mouse: effect of standard vivarium lighting on retinal pathology during aging","volume":"135","author":"Bell","year":"2015","journal-title":"Exp. Eye Res."},{"key":"10.1016\/j.jbc.2026.111261_bib75","doi-asserted-by":"crossref","first-page":"2921","DOI":"10.1167\/iovs.12-9662","article-title":"The Rd8 mutation of the Crb1 gene is present in vendor lines of C57BL\/6N mice and embryonic stem cells, and confounds ocular induced mutant phenotypes","volume":"53","author":"Mattapallil","year":"2012","journal-title":"Invest Ophthalmol. Vis. Sci."},{"key":"10.1016\/j.jbc.2026.111261_bib76","doi-asserted-by":"crossref","first-page":"5190","DOI":"10.1167\/iovs.12-9672","article-title":"Fundus autofluorescence findings in a mouse model of retinal detachment","volume":"53","author":"Secondi","year":"2012","journal-title":"Invest Ophthalmol. Vis. Sci."},{"key":"10.1016\/j.jbc.2026.111261_bib77","first-page":"908","article-title":"Anti-vascular endothelial growth factor acts on retinal microglia\/macrophage activation in a rat model of ocular inflammation","volume":"20","author":"Couturier","year":"2014","journal-title":"Mol. Vis."},{"key":"10.1016\/j.jbc.2026.111261_bib78","doi-asserted-by":"crossref","first-page":"723","DOI":"10.1016\/j.immuni.2019.02.007","article-title":"Microglial function is distinct in different anatomical locations during retinal homeostasis and degeneration","volume":"50","author":"O'Koren","year":"2019","journal-title":"Immunity"},{"key":"10.1016\/j.jbc.2026.111261_bib79","doi-asserted-by":"crossref","first-page":"12","DOI":"10.1186\/s13064-019-0137-x","article-title":"Microglia in the developing retina","volume":"14","author":"Li","year":"2019","journal-title":"Neural. Dev."},{"key":"10.1016\/j.jbc.2026.111261_bib80","doi-asserted-by":"crossref","DOI":"10.1155\/2023\/6815504","article-title":"Compendium of clinical variant classification for 2,246 unique ABCA4 variants to clarify variant pathogenicity in Stargardt disease using a modified ACMG\/AMP framework","volume":"2023","author":"Cornelis","year":"2023","journal-title":"Hum. Mutat."},{"key":"10.1016\/j.jbc.2026.111261_bib81","doi-asserted-by":"crossref","first-page":"1293","DOI":"10.1093\/hmg\/ddab122","article-title":"Cis-acting modifiers in the ABCA4 locus contribute to the penetrance of the major disease-causing variant in Stargardt disease","volume":"30","author":"Lee","year":"2021","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.jbc.2026.111261_bib82","doi-asserted-by":"crossref","first-page":"2305","DOI":"10.1167\/iovs.17-23364","article-title":"Correlating the expression and functional activity of ABCA4 disease variants with the phenotype of patients with Stargardt disease","volume":"59","author":"Garces","year":"2018","journal-title":"Invest Ophthalmol. Vis. Sci."},{"key":"10.1016\/j.jbc.2026.111261_bib83","doi-asserted-by":"crossref","first-page":"340","DOI":"10.1016\/j.ajo.2020.08.042","article-title":"Quantitative fundus autofluorescence in ABCA4-Related retinopathy -Functional relevance and genotype-phenotype correlation","volume":"222","author":"Muller","year":"2021","journal-title":"Am. J. Ophthalmol."},{"key":"10.1016\/j.jbc.2026.111261_bib84","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1167\/iovs.64.12.33","article-title":"ABCA4 variant c.5714+5G>A in trans with null alleles results in primary RPE damage","volume":"64","author":"Sajovic","year":"2023","journal-title":"Invest Ophthalmol. Vis. Sci."},{"key":"10.1016\/j.jbc.2026.111261_bib85","doi-asserted-by":"crossref","first-page":"676","DOI":"10.1038\/nmeth.2019","article-title":"Fiji: an open-source platform for biological-image analysis","volume":"9","author":"Schindelin","year":"2012","journal-title":"Nat. Methods"},{"key":"10.1016\/j.jbc.2026.111261_bib86","first-page":"1185","article-title":"DHA does not protect ELOVL4 transgenic mice from retinal degeneration","volume":"15","author":"Li","year":"2009","journal-title":"Mol. Vis."},{"key":"10.1016\/j.jbc.2026.111261_bib87","doi-asserted-by":"crossref","DOI":"10.1016\/j.jlr.2024.100498","article-title":"APOE genotype dictates lipidomic signatures in primary human hepatocytes","volume":"65","author":"Almeida","year":"2024","journal-title":"J. Lipid Res."},{"key":"10.1016\/j.jbc.2026.111261_bib88","doi-asserted-by":"crossref","first-page":"210","DOI":"10.1038\/s42003-023-04573-z","article-title":"Lysosomal phospholipase A2 contributes to the biosynthesis of the atypical late endosome lipid bis(monoacylglycero)phosphate","volume":"6","author":"Chen","year":"2023","journal-title":"Commun. Biol."}],"container-title":["Journal of Biological Chemistry"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0021925826001316?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0021925826001316?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2026,4,16]],"date-time":"2026-04-16T23:17:19Z","timestamp":1776381439000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0021925826001316"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,3]]},"references-count":89,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2026,3]]}},"alternative-id":["S0021925826001316"],"URL":"https:\/\/doi.org\/10.1016\/j.jbc.2026.111261","relation":{},"ISSN":["0021-9258"],"issn-type":[{"value":"0021-9258","type":"print"}],"subject":[],"published":{"date-parts":[[2026,3]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"More insights from Abca4\u2212\/\u2212 mouse models of recessive Stargardt disease","name":"articletitle","label":"Article Title"},{"value":"Journal of Biological Chemistry","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.jbc.2026.111261","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2026 The Authors. Published by Elsevier Inc on behalf of American Society for Biochemistry and Molecular Biology.","name":"copyright","label":"Copyright"}],"article-number":"111261"}}