{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,21]],"date-time":"2026-10-21T11:20:09Z","timestamp":1792581609181,"version":"4.5.0"},"reference-count":33,"publisher":"Association for Research in Vision and Ophthalmology (ARVO)","issue":"6","license":[{"start":{"date-parts":[[2026,6,12]],"date-time":"2026-06-12T00:00:00Z","timestamp":1781222400000},"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":[[2026,6,12]]},"DOI":"10.1167\/iovs.67.6.23","type":"journal-article","created":{"date-parts":[[2026,6,12]],"date-time":"2026-06-12T13:30:58Z","timestamp":1781271058000},"page":"23","source":"Crossref","is-referenced-by-count":2,"title":["Directional Analysis of Residual RPE Area Loss in the Natural History of Choroideremia"],"prefix":"10.1167","volume":"67","author":[{"given":"Shinichiro","family":"Chujo","sequence":"first","affiliation":[{"name":"Doheny Eye Institute, Pasadena, California, United States"},{"name":"Department of Ophthalmology David Geffen School of Medicine, University of California Los Angeles, Los Angeles, California, United States"},{"name":"Department of Ophthalmology, Mie University Graduate School of Medicine, Tsu City, Mie, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rouzbeh","family":"Abbasgholizadeh","sequence":"additional","affiliation":[{"name":"Doheny Eye Institute, Pasadena, California, United States"},{"name":"Department of Ophthalmology David Geffen School of Medicine, University of California Los Angeles, Los Angeles, California, United States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mai","family":"Alhelaly","sequence":"additional","affiliation":[{"name":"Doheny Eye Institute, Pasadena, California, United States"},{"name":"Department of Ophthalmology David Geffen School of Medicine, University of California Los Angeles, Los Angeles, California, United States"},{"name":"Ophthalmology Department, Tanta University, Tanta, Gharbia, Egypt"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Muneeswar Gupta","family":"Nittala","sequence":"additional","affiliation":[{"name":"Doheny Eye Institute, Pasadena, California, United States"},{"name":"Department of Ophthalmology David Geffen School of Medicine, University of California Los Angeles, Los Angeles, California, United States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alberto","family":"Quarta","sequence":"additional","affiliation":[{"name":"Doheny Eye Institute, Pasadena, California, United States"},{"name":"Department of Ophthalmology David Geffen School of Medicine, University of California Los Angeles, Los Angeles, California, United States"},{"name":"Department of Neurosciences, Imaging and Clinical Sciences, University \u201cG. d'Annunzio\u201d Chieti-Pescara, Chieti, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yu-Chien","family":"Chung","sequence":"additional","affiliation":[{"name":"Doheny Eye Institute, Pasadena, California, United States"},{"name":"Department of Ophthalmology David Geffen School of Medicine, University of California Los Angeles, Los Angeles, California, United States"},{"name":"Department of Ophthalmology, Fu Jen Catholic University Hospital, Fu Jen Catholic University, New Taipei City, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hyunduck","family":"Kwak","sequence":"additional","affiliation":[{"name":"Doheny Eye Institute, Pasadena, California, United States"},{"name":"Department of Ophthalmology David Geffen School of Medicine, University of California Los Angeles, Los Angeles, California, United States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ceren","family":"Soylu","sequence":"additional","affiliation":[{"name":"Doheny Eye Institute, Pasadena, California, United States"},{"name":"Department of Ophthalmology David Geffen School of Medicine, University of California Los Angeles, Los Angeles, California, United States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Raiyna","family":"Rattu","sequence":"additional","affiliation":[{"name":"Doheny Eye Institute, Pasadena, California, United States"},{"name":"Department of Ophthalmology David Geffen School of Medicine, University of California Los Angeles, Los Angeles, California, United States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jianfeng","family":"Huang","sequence":"additional","affiliation":[{"name":"Doheny Eye Institute, Pasadena, California, United States"},{"name":"Department of Ophthalmology David Geffen School of Medicine, University of California Los Angeles, Los Angeles, California, United States"},{"name":"Beijing Hospital, National Center of Gerontology, Institute of Geriatric Medicine, Chinese Academy of Medical Sciences, Beijing, P.R. China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giulia","family":"Corradetti","sequence":"additional","affiliation":[{"name":"Doheny Eye Institute, Pasadena, California, United States"},{"name":"Department of Ophthalmology David Geffen School of Medicine, University of California Los Angeles, Los Angeles, California, United States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Swetha","family":"Velaga","sequence":"additional","affiliation":[{"name":"Doheny Eye Institute, Pasadena, California, United States"},{"name":"Department of Ophthalmology David Geffen School of Medicine, University of California Los Angeles, Los Angeles, California, United States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paolo","family":"Forte","sequence":"additional","affiliation":[{"name":"Doheny Eye Institute, Pasadena, California, United States"},{"name":"Department of Ophthalmology David Geffen School of Medicine, University of California Los Angeles, Los Angeles, California, United States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Akihito","family":"Uji","sequence":"additional","affiliation":[{"name":"Uji Eye Clinic, Yokkaichi City, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Srinivas R.","family":"Sadda","sequence":"additional","affiliation":[{"name":"Doheny Eye Institute, Pasadena, California, United States"},{"name":"Department of Ophthalmology David Geffen School of Medicine, University of California Los Angeles, Los Angeles, California, United States"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"27","reference":[{"issue":"2","key":"bib1","doi-asserted-by":"publisher","first-page":"110","DOI":"10.1002\/(SICI)1098-1004(1997)9:2&lt;110::AID-HUMU2&gt;3.0.CO;2-D","article-title":"Molecular basis of choroideremia (CHM): mutations involving the Rab escort protein-1 (REP-1) gene","volume":"9","author":"van den Hurk","year":"1997","journal-title":"Hum Mutat"},{"key":"bib2","first-page":"2515841418817490","article-title":"Choroideremia: from genetic and clinical phenotyping to gene therapy and future treatments","volume":"10","author":"Mitsios","year":"2018","journal-title":"Ther Adv Ophthalmol"},{"issue":"11","key":"bib3","doi-asserted-by":"publisher","first-page":"2059","DOI":"10.1097\/IAE.0000000000002553","article-title":"Choroideremia: retinal degeneration with an unmet need","volume":"39","author":"Pennesi","year":"2019","journal-title":"Retina"},{"key":"bib4","doi-asserted-by":"publisher","first-page":"65","DOI":"10.1016\/j.ajo.2018.09.012","article-title":"Choroideremia gene therapy phase 2 clinical trial: 24-month results","volume":"197","author":"Lam","year":"2019","journal-title":"Am J Ophthalmol"},{"issue":"10","key":"bib5","doi-asserted-by":"publisher","first-page":"1507","DOI":"10.1038\/s41591-018-0185-5","article-title":"Beneficial effects on vision in patients undergoing retinal gene therapy for choroideremia","volume":"24","author":"Xue","year":"2018","journal-title":"Nat Med"},{"key":"bib6","doi-asserted-by":"publisher","first-page":"130","DOI":"10.1016\/j.ajo.2018.06.011","article-title":"Two-year results after AAV2-mediated gene therapy for choroideremia: the Alberta experience","volume":"193","author":"Dimopoulos","year":"2018","journal-title":"Am J Ophthalmol"},{"key":"bib7","doi-asserted-by":"publisher","first-page":"1247","DOI":"10.1001\/jamaophthalmol.2019.3278","article-title":"Efficacy and safety of retinal gene therapy using an adeno-associated virus vector for patients with choroideremia: a randomized clinical trial","volume":"137","author":"Fischer","year":"2019","journal-title":"JAMA Ophthalmol"},{"issue":"10","key":"bib8","doi-asserted-by":"publisher","first-page":"888","DOI":"10.1016\/j.oret.2019.05.002","article-title":"Visual function at the atrophic border in choroideremia assessed with adaptive optics microperimetry","volume":"3","author":"Tuten","year":"2019","journal-title":"Ophthalmol Retina"},{"issue":"7","key":"bib9","doi-asserted-by":"publisher","first-page":"933","DOI":"10.1136\/bjophthalmol-2018-312620","article-title":"Macular spatial distribution of preserved autofluorescence in patients with choroideremia","volume":"103","author":"Hariri","year":"2019","journal-title":"Br J Ophthalmol"},{"issue":"14","key":"bib10","doi-asserted-by":"publisher","first-page":"10","DOI":"10.1167\/iovs.62.14.10","article-title":"An in silico model for RPE loss patterns in choroideremia","volume":"62","author":"Young","year":"2021","journal-title":"Invest Ophthalmol Vis Sci"},{"issue":"10","key":"bib11","doi-asserted-by":"publisher","first-page":"4498","DOI":"10.1167\/iovs.15-18362","article-title":"A qualitative and quantitative assessment of fundus autofluorescence patterns in patients with choroideremia","volume":"57","author":"Jolly","year":"2016","journal-title":"Invest Ophthalmol Vis Sci"},{"key":"bib12","doi-asserted-by":"publisher","first-page":"35","DOI":"10.1016\/j.ajo.2024.01.022","article-title":"A prospective, observational, non-interventional clinical study of participants with choroideremia: the NIGHT study","volume":"263","author":"MacLaren","year":"2024","journal-title":"Am J Ophthalmol"},{"issue":"1","key":"bib13","doi-asserted-by":"publisher","first-page":"29","DOI":"10.1186\/s12886-022-02250-z","article-title":"Bilateral visual acuity decline in males with choroideremia: a pooled, cross-sectional meta-analysis","volume":"22","author":"Bozkaya","year":"2022","journal-title":"BMC Ophthalmol"},{"key":"bib14","doi-asserted-by":"publisher","first-page":"1679","DOI":"10.1007\/s00417-019-04368-1","article-title":"Directional kinetics analysis of the progression of geographic atrophy","volume":"257","author":"Uji","year":"2019","journal-title":"Graefes Arch Clin Exp Ophthalmol"},{"key":"bib15","doi-asserted-by":"publisher","first-page":"1858","DOI":"10.1109\/TPAMI.2008.113","article-title":"Parametric image alignment using enhanced correlation coefficient maximization","volume":"30","author":"Evangelidis","year":"2008","journal-title":"IEEE Trans Pattern Anal Mach Intell"},{"key":"bib16","doi-asserted-by":"publisher","first-page":"110","DOI":"10.1016\/j.ajo.2017.05.002","article-title":"Measurement and reproducibility of preserved ellipsoid zone area and preserved retinal pigment epithelium area in eyes with choroideremia","volume":"179","author":"Hariri","year":"2017","journal-title":"Am J Ophthalmol"},{"issue":"12","key":"bib17","doi-asserted-by":"publisher","first-page":"1068","DOI":"10.3390\/cells13121068","article-title":"Reduced retinal pigment epithelial autophagy due to loss of Rab12 prenylation in a human iPSC-RPE model of choroideremia","volume":"13","author":"Raeker","year":"2024","journal-title":"Cells"},{"issue":"4","key":"bib18","doi-asserted-by":"publisher","first-page":"e0151943","DOI":"10.1371\/journal.pone.0151943","article-title":"A comprehensive analysis of choroideremia: from genetic characterization to clinical practice","volume":"11","author":"Sanchez-Alcudia","year":"2016","journal-title":"PLoS One"},{"issue":"6","key":"bib19","doi-asserted-by":"publisher","first-page":"1049","DOI":"10.1016\/0092-8674(92)90253-9","article-title":"Purification of component A of Rab geranylgeranyl transferase: possible identity with the choroideremia gene product","volume":"70","author":"Seabra","year":"1992","journal-title":"Cell"},{"issue":"2","key":"bib20","doi-asserted-by":"publisher","first-page":"101","DOI":"10.1080\/13816810490514333","article-title":"Rab escort protein 1 (REP1) in intracellular traffic: a functional and pathophysiological overview","volume":"25","author":"Preising","year":"2004","journal-title":"Ophthalmic Genet"},{"issue":"4","key":"bib21","doi-asserted-by":"publisher","first-page":"497","DOI":"10.1002\/cne.902920402","article-title":"Human photoreceptor topography","volume":"292","author":"Curcio","year":"1990","journal-title":"J Comp Neurol"},{"issue":"3","key":"bib22","doi-asserted-by":"publisher","first-page":"4","DOI":"10.1167\/iovs.65.3.4","article-title":"Age-related macular degeneration, a mathematically tractable disease","volume":"65","author":"Curcio","year":"2024","journal-title":"Invest Ophthalmol Vis Sci"},{"issue":"1","key":"bib23","doi-asserted-by":"publisher","first-page":"196","DOI":"10.1186\/s12886-020-01467-0","article-title":"Functionally validated imaging endpoints in the Alabama study on early age-related macular degeneration 2 (ALSTAR2): design and methods","volume":"20","author":"Curcio","year":"2020","journal-title":"BMC Ophthalmol"},{"issue":"2","key":"bib24","first-page":"229","article-title":"A histopathologic study of a choroideremia carrier","volume":"31","author":"Flannery","year":"1990","journal-title":"Invest Ophthalmol Vis Sci"},{"issue":"10","key":"bib25","doi-asserted-by":"publisher","first-page":"6381","DOI":"10.1167\/iovs.13-13454","article-title":"High-resolution adaptive optics retinal imaging of cellular structure in choroideremia","volume":"55","author":"Morgan","year":"2014","journal-title":"Invest Ophthalmol Vis Sci"},{"issue":"8","key":"bib26","doi-asserted-by":"publisher","first-page":"1107","DOI":"10.1001\/archopht.125.8.1107","article-title":"Clinical and functional findings in choroideremia due to complete deletion of the CHM gene","volume":"125","author":"Mura","year":"2007","journal-title":"Arch Ophthalmol"},{"issue":"6","key":"bib27","doi-asserted-by":"publisher","first-page":"697","DOI":"10.1001\/jamaophthalmol.2016.0874","article-title":"Optical coherence tomography angiography in choroideremia: correlating choriocapillaris loss with overlying degeneration","volume":"134","author":"Jain","year":"2016","journal-title":"JAMA Ophthalmol"},{"issue":"4","key":"bib28","doi-asserted-by":"publisher","first-page":"711","DOI":"10.1016\/S0161-6420(00)00643-6","article-title":"Evaluation of retinal photoreceptors and pigment epithelium in a female carrier of choroideremia","volume":"108","author":"Syed","year":"2001","journal-title":"Ophthalmology"},{"issue":"9","key":"bib29","doi-asserted-by":"publisher","first-page":"4113","DOI":"10.1167\/iovs.06-0424","article-title":"Remodeling of the human retina in choroideremia: Rab escort protein 1 (REP-1) mutations","volume":"47","author":"Jacobson","year":"2006","journal-title":"Invest Ophthalmol Vis Sci"},{"issue":"13","key":"bib30","doi-asserted-by":"publisher","first-page":"9581","DOI":"10.1167\/iovs.11-8554","article-title":"Transition zones between healthy and diseased retina in choroideremia (CHM) and Stargardt disease (STGD) as compared to retinitis pigmentosa (RP)","volume":"52","author":"Lazow","year":"2011","journal-title":"Invest Ophthalmol Vis Sci"},{"issue":"12","key":"bib31","doi-asserted-by":"publisher","first-page":"1200","DOI":"10.1016\/j.oret.2024.06.011","article-title":"Retinal characteristics of female choroideremia carriers: multimodal imaging, microperimetry, and genetics","volume":"8","author":"Gocuk","year":"2024","journal-title":"Ophthalmol Retina"},{"issue":"9","key":"bib32","doi-asserted-by":"publisher","first-page":"24","DOI":"10.1167\/tvst.12.9.24","article-title":"Correlation between fundus autofluorescence pattern and retinal function on microperimetry in choroideremia","volume":"12","author":"Poli","year":"2023","journal-title":"Transl Vis Sci Technol"},{"issue":"12","key":"bib33","doi-asserted-by":"publisher","first-page":"5575","DOI":"10.1167\/iovs.17-22486","article-title":"Characterizing the natural history of visual function in choroideremia using microperimetry and multimodal retinal imaging","volume":"58","author":"Jolly","year":"2017","journal-title":"Invest Ophthalmol Vis Sci"}],"container-title":["Investigative Ophthalmology &amp; Visual Science"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/iovs.arvojournals.org\/article.aspx?doi=10.1167\/iovs.67.6.23","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T21:32:16Z","timestamp":1791927136000},"score":1,"resource":{"primary":{"URL":"http:\/\/iovs.arvojournals.org\/article.aspx?doi=10.1167\/iovs.67.6.23"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,6,12]]},"references-count":33,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2026,6,1]]}},"URL":"https:\/\/doi.org\/10.1167\/iovs.67.6.23","relation":{},"ISSN":["1552-5783"],"issn-type":[{"value":"1552-5783","type":"print"}],"subject":[],"published":{"date-parts":[[2026,6,12]]}}}