{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T22:00:37Z","timestamp":1791928837084,"version":"4.3.4"},"reference-count":50,"publisher":"Association for Research in Vision and Ophthalmology (ARVO)","issue":"14","license":[{"start":{"date-parts":[[2024,12,11]],"date-time":"2024-12-11T00:00:00Z","timestamp":1733875200000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Invest. Ophthalmol. Vis. Sci."],"published-print":{"date-parts":[[2024,12,11]]},"DOI":"10.1167\/iovs.65.14.24","type":"journal-article","created":{"date-parts":[[2024,12,11]],"date-time":"2024-12-11T15:39:23Z","timestamp":1733931563000},"page":"24","source":"Crossref","is-referenced-by-count":1,"title":["Sphingolipid Levels and Processing of the Retinyl Chromophore in the Retina of a Mouse Model of Niemann-Pick Disease"],"prefix":"10.1167","volume":"65","author":[{"given":"Bushra","family":"Rahman","sequence":"first","affiliation":[{"name":"Department of Ophthalmology, Medical University of South Carolina, Charleston, South Carolina, United States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David M. G.","family":"Anderson","sequence":"additional","affiliation":[{"name":"Mass Spectrometry Research Center and Department of Biochemistry, Vanderbilt University School of Medicine, Nashville, Tennessee, Unites States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chunhe","family":"Chen","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology, Medical University of South Carolina, Charleston, South Carolina, United States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jian","family":"Liu","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology, Medical University of South Carolina, Charleston, South Carolina, United States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lukasz G.","family":"Migas","sequence":"additional","affiliation":[{"name":"Delft Center for Systems and Control (DCSC), Delft University of Technology, Delft, Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Raf","family":"Van de Plas","sequence":"additional","affiliation":[{"name":"Delft Center for Systems and Control (DCSC), Delft University of Technology, Delft, Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kevin L.","family":"Schey","sequence":"additional","affiliation":[{"name":"Mass Spectrometry Research Center and Department of Biochemistry, Vanderbilt University School of Medicine, Nashville, Tennessee, Unites States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masahiro","family":"Kono","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology, Medical University of South Carolina, Charleston, South Carolina, United States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jie","family":"Fan","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology, Medical University of South Carolina, Charleston, South Carolina, United States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yiannis","family":"Koutalos","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology, Medical University of South Carolina, Charleston, South Carolina, United States"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"27","reference":[{"key":"bib1","doi-asserted-by":"publisher","first-page":"169","DOI":"10.1016\/S0167-4943(02)00005-5","article-title":"What is lipofuscin? Defining characteristics and differentiation from other autofluorescent lysosomal storage bodies","volume":"34","author":"Katz","year":"2002","journal-title":"Arch Gerontol Geriatr"},{"key":"bib2","doi-asserted-by":"publisher","first-page":"1400","DOI":"10.1016\/j.biocel.2003.08.009","article-title":"Lipofuscin","volume":"36","author":"Terman","year":"2004","journal-title":"Int J Biochem Cell Biol"},{"key":"bib3","first-page":"583","article-title":"Lipofuscin and melanin of human retinal pigment epithelium. Fluorescence, enzyme cytochemical, and ultrastructural studies","volume":"17","author":"Feeney","year":"1978","journal-title":"Invest Ophthalmol Vis Sci"},{"key":"bib4","doi-asserted-by":"publisher","first-page":"595","DOI":"10.1016\/j.exer.2005.01.007","article-title":"RPE lipofuscin and its role in retinal pathobiology","volume":"80","author":"Sparrow","year":"2005","journal-title":"Exp Eye Res"},{"key":"bib5","doi-asserted-by":"publisher","first-page":"554","DOI":"10.1038\/nrm1423","article-title":"The cell biology of lysosomal storage disorders","volume":"5","author":"Futerman","year":"2004","journal-title":"Nat Rev Mol Cell Biol"},{"key":"bib6","doi-asserted-by":"publisher","first-page":"107851","DOI":"10.1016\/j.neuropharm.2019.107851","article-title":"Addressing neurodegeneration in lysosomal storage disorders: Advances in Niemann Pick diseases","volume":"171","author":"Toledano-Zaragoza","year":"2020","journal-title":"Neuropharmacology"},{"key":"bib7","doi-asserted-by":"publisher","first-page":"27","DOI":"10.1016\/j.ymgme.2016.12.008","article-title":"Types A and B Niemann-Pick disease","volume":"120","author":"Schuchman","year":"2017","journal-title":"Mol Genet Metab"},{"key":"bib8","doi-asserted-by":"publisher","first-page":"e0133032","DOI":"10.1371\/journal.pone.0133032","article-title":"Lack of Acid Sphingomyelinase Induces Age-Related Retinal Degeneration","volume":"10","author":"Wu","year":"2015","journal-title":"PLoS One"},{"key":"bib9","doi-asserted-by":"publisher","first-page":"e1","DOI":"10.1016\/bs.pmbts.2015.07.022","article-title":"The 11-cis retinal origins of lipofuscin in the retina","volume":"134","author":"Adler","year":"2015","journal-title":"Prog Mol Biol Transl Sci"},{"key":"bib10","doi-asserted-by":"publisher","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":"bib11","doi-asserted-by":"publisher","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":"bib12","doi-asserted-by":"publisher","first-page":"724","DOI":"10.1038\/361724a0","article-title":"Retinal age pigments generated by self-assembling lysosomotropic detergents","volume":"361","author":"Eldred","year":"1993","journal-title":"Nature"},{"key":"bib13","doi-asserted-by":"publisher","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 USA"},{"key":"bib14","doi-asserted-by":"publisher","first-page":"3842","DOI":"10.1073\/pnas.052025899","article-title":"The lipofuscin component A2E selectively inhibits phagolysosomal degradation of photoreceptor phospholipid by the retinal pigment epithelium","volume":"99","author":"Finnemann","year":"2002","journal-title":"Proc Natl Acad Sci USA"},{"key":"bib15","doi-asserted-by":"publisher","first-page":"11026","DOI":"10.1073\/pnas.0702504104","article-title":"The lipofuscin fluorophore A2E perturbs cholesterol metabolism in retinal pigment epithelial cells","volume":"104","author":"Lakkaraju","year":"2007","journal-title":"Proc Natl Acad Sci USA"},{"key":"bib16","doi-asserted-by":"publisher","first-page":"7183","DOI":"10.1074\/jbc.M108981200","article-title":"Biosynthetic studies of A2E, a major fluorophore of retinal pigment epithelial lipofuscin","volume":"277","author":"Ben-Shabat","year":"2002","journal-title":"J Biol Chem"},{"key":"bib17","doi-asserted-by":"publisher","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":"bib18","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1167\/iovs.62.2.1","article-title":"Relative contributions of all-trans and 11-Cis retinal to formation of lipofuscin and A2E accumulating in mouse retinal pigment epithelium","volume":"62","author":"Boyer","year":"2021","journal-title":"Invest Ophthalmol Vis Sci"},{"key":"bib19","doi-asserted-by":"publisher","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 USA"},{"key":"bib20","doi-asserted-by":"publisher","first-page":"49","DOI":"10.1016\/S1350-9462(00)00014-8","article-title":"Vertebrate photoreceptors","volume":"20","author":"Ebrey","year":"2001","journal-title":"Prog Retin Eye Res"},{"key":"bib21","doi-asserted-by":"publisher","first-page":"230","DOI":"10.1126\/science.162.3850.230","article-title":"Molecular basis of visual excitation","volume":"162","author":"Wald","year":"1968","journal-title":"Science"},{"key":"bib22","doi-asserted-by":"publisher","first-page":"307","DOI":"10.1016\/j.preteyeres.2004.03.001","article-title":"Dark adaptation and the retinoid cycle of vision","volume":"23","author":"Lamb","year":"2004","journal-title":"Prog Retin Eye Res"},{"key":"bib23","first-page":"337","article-title":"Biochemistry of visual pigment regeneration: the Friedenwald lecture","volume":"41","author":"Saari","year":"2000","journal-title":"Invest Ophthalmol Vis Sci"},{"key":"bib24","doi-asserted-by":"publisher","first-page":"6907","DOI":"10.1073\/pnas.87.17.6907","article-title":"Interphotoreceptor retinoid-binding protein promotes rhodopsin regeneration in toad photoreceptors","volume":"87","author":"Okajima","year":"1990","journal-title":"Proc Natl Acad Sci USA"},{"key":"bib25","doi-asserted-by":"publisher","first-page":"24662","DOI":"10.1074\/jbc.M112.354514","article-title":"Reduction of all-trans-retinal in vertebrate rod photoreceptors requires the combined action of RDH8 and RDH12","volume":"287","author":"Chen","year":"2012","journal-title":"J Biol Chem"},{"key":"bib26","doi-asserted-by":"publisher","first-page":"18822","DOI":"10.1074\/jbc.M501757200","article-title":"Role of photoreceptor-specific retinol dehydrogenase in the retinoid cycle in vivo","volume":"280","author":"Maeda","year":"2005","journal-title":"J Biol Chem"},{"key":"bib27","doi-asserted-by":"publisher","first-page":"11034","DOI":"10.1074\/jbc.275.15.11034","article-title":"Identification and characterization of all-trans-retinol dehydrogenase from photoreceptor outer segments, the visual cycle enzyme that reduces all-trans-retinal to all-trans-retinol","volume":"275","author":"Rattner","year":"2000","journal-title":"J Biol Chem"},{"key":"bib28","doi-asserted-by":"publisher","first-page":"149","DOI":"10.1111\/j.1471-4159.1970.tb02195.x","article-title":"Metabolism of glucose and reduction of retinaldehyde in retinal photoreceptors","volume":"17","author":"Futterman","year":"1970","journal-title":"J Neurochem"},{"key":"bib29","doi-asserted-by":"publisher","first-page":"1415","DOI":"10.1111\/j.1471-4159.2008.05497.x","article-title":"Accumulation of bis(monoacylglycero)phosphate and gangliosides in mouse models of neuronal ceroid lipofuscinosis","volume":"106","author":"Jabs","year":"2008","journal-title":"J Neurochem"},{"key":"bib30","doi-asserted-by":"publisher","first-page":"287","DOI":"10.1007\/BF02531203","article-title":"Accumulation of a glycerolphospholipid in classical niemann-pick disease","volume":"3","author":"Rouser","year":"1968","journal-title":"Lipids"},{"key":"bib31","doi-asserted-by":"publisher","first-page":"726","DOI":"10.1016\/j.bbamcr.2008.11.014","article-title":"Secondary lipid accumulation in lysosomal disease","volume":"1793","author":"Walkley","year":"2009","journal-title":"Biochim Biophys Acta"},{"key":"bib32","doi-asserted-by":"publisher","first-page":"288","DOI":"10.1038\/ng0795-288","article-title":"Acid sphingomyelinase deficient mice: a model of types A and B Niemann-Pick disease","volume":"10","author":"Horinouchi","year":"1995","journal-title":"Nat Genet"},{"key":"bib33","doi-asserted-by":"publisher","first-page":"1519","DOI":"10.1074\/jbc.M113.511295","article-title":"Mitochondria contribute to NADPH generation in mouse rod photoreceptors","volume":"289","author":"Adler","year":"2014","journal-title":"J Biol Chem"},{"key":"bib34","doi-asserted-by":"publisher","first-page":"3589","DOI":"10.1167\/iovs.08-3336","article-title":"Formation of all-trans retinol after visual pigment bleaching in mouse photoreceptors","volume":"50","author":"Chen","year":"2009","journal-title":"Invest Ophthalmol Vis Sci"},{"key":"bib35","doi-asserted-by":"publisher","first-page":"153","DOI":"10.1085\/jgp.200609557","article-title":"Visual cycle: dependence of retinol production and removal on photoproduct decay and cell morphology","volume":"128","author":"Ala-Laurila","year":"2006","journal-title":"J Gen Physiol"},{"key":"bib36","doi-asserted-by":"publisher","first-page":"8669","DOI":"10.1021\/bi7004619","article-title":"Interphotoreceptor retinoid-binding protein is the physiologically relevant carrier that removes retinol from rod photoreceptor outer segments","volume":"46","author":"Wu","year":"2007","journal-title":"Biochemistry"},{"key":"bib37","doi-asserted-by":"publisher","first-page":"2732","DOI":"10.1021\/bi00432a055","article-title":"Regeneration of bovine and octopus opsins in situ with natural and artificial retinals","volume":"28","author":"Koutalos","year":"1989","journal-title":"Biochemistry"},{"key":"bib38","doi-asserted-by":"publisher","first-page":"14552","DOI":"10.1021\/acs.analchem.9b03612","article-title":"High-performance molecular imaging with MALDI trapped ion-mobility time-of-flight (timsTOF) mass spectrometry","volume":"91","author":"Spraggins","year":"2019","journal-title":"Anal Chem"},{"key":"bib39","doi-asserted-by":"publisher","first-page":"736","DOI":"10.1073\/pnas.96.2.736","article-title":"Morphological, physiological, and biochemical changes in rhodopsin knockout mice","volume":"96","author":"Lem","year":"1999","journal-title":"Proc Natl Acad Sci USA"},{"key":"bib40","doi-asserted-by":"publisher","first-page":"2426","DOI":"10.1021\/jasms.0c00119","article-title":"Lipid landscape of the human retina and supporting tissues revealed by high-resolution imaging mass spectrometry","volume":"31","author":"Anderson","year":"2020","journal-title":"J Am Soc Mass Spectrom"},{"key":"bib41","doi-asserted-by":"publisher","first-page":"1060","DOI":"10.1046\/j.1471-4159.1994.63031060.x","article-title":"Accurate differentiation of neuronopathic and nonneuronopathic forms of Niemann-Pick disease by evaluation of the effective residual lysosomal sphingomyelinase activity in intact cells","volume":"63","author":"Graber","year":"1994","journal-title":"J Neurochem"},{"key":"bib42","doi-asserted-by":"publisher","first-page":"174","DOI":"10.1016\/S0002-9394(14)75945-8","article-title":"Ocular manifestations of group A Niemann-Pick disease","volume":"85","author":"Walton","year":"1978","journal-title":"Am J Ophthalmol"},{"key":"bib43","doi-asserted-by":"publisher","first-page":"1244","DOI":"10.1001\/jamaophthalmol.2013.2374","article-title":"Inner macular hyperreflectivity demonstrated by optical coherence tomography in niemann-pick disease","volume":"131","author":"Rudich","year":"2013","journal-title":"JAMA Ophthalmol"},{"key":"bib44","first-page":"366","article-title":"The ocular pathology of type A Niemann-Pick disease. A light and electron microscopic study","volume":"12","author":"Robb","year":"1973","journal-title":"Invest Ophthalmol"},{"key":"bib45","doi-asserted-by":"publisher","first-page":"121","DOI":"10.1016\/j.exer.2017.02.007","article-title":"All-trans retinal levels and formation of lipofuscin precursors after bleaching in rod photoreceptors from wild type and Abca4(-\/-) mice","volume":"155","author":"Adler","year":"2017","journal-title":"Exp Eye Res"},{"key":"bib46","doi-asserted-by":"publisher","first-page":"17352","DOI":"10.1038\/s41598-017-17402-1","article-title":"Bis(monoacylglycero)phosphate lipids in the retinal pigment epithelium implicate lysosomal\/endosomal dysfunction in a model of Stargardt disease and human retinas","volume":"7","author":"Anderson","year":"2017","journal-title":"Sci Rep"},{"key":"bib47","doi-asserted-by":"publisher","first-page":"18431","DOI":"10.1074\/jbc.271.31.18431","article-title":"Zn2+-stimulated sphingomyelinase is secreted by many cell types and is a product of the acid sphingomyelinase gene","volume":"271","author":"Schissel","year":"1996","journal-title":"J Biol Chem"},{"key":"bib48","doi-asserted-by":"publisher","first-page":"109250","DOI":"10.1016\/j.exer.2022.109250","article-title":"Sphingomyelinases in retinas and optic nerve heads: Effects of ocular hypertension and ischemia","volume":"224","author":"Fan","year":"2022","journal-title":"Exp Eye Res"},{"key":"bib49","doi-asserted-by":"publisher","first-page":"27","DOI":"10.1139\/o03-091","article-title":"Acid and neutral sphingomyelinases: roles and mechanisms of regulation","volume":"82","author":"Marchesini","year":"2004","journal-title":"Biochem Cell Biol"},{"key":"bib50","doi-asserted-by":"publisher","first-page":"836","DOI":"10.1016\/j.cellsig.2009.01.026","article-title":"Roles and regulation of secretory and lysosomal acid sphingomyelinase","volume":"21","author":"Jenkins","year":"2009","journal-title":"Cell Signal"}],"container-title":["Investigative Ophthalmology &amp; Visual Science"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/iovs.arvojournals.org\/article.aspx?articleid=2802350","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T21:31:50Z","timestamp":1791927110000},"score":1,"resource":{"primary":{"URL":"https:\/\/iovs.arvojournals.org\/article.aspx?articleid=2802350"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,12,11]]},"references-count":50,"journal-issue":{"issue":"14","published-print":{"date-parts":[[2024,12,2]]}},"URL":"https:\/\/doi.org\/10.1167\/iovs.65.14.24","relation":{},"ISSN":["1552-5783"],"issn-type":[{"value":"1552-5783","type":"print"}],"subject":[],"published":{"date-parts":[[2024,12,11]]}}}