{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T22:01:05Z","timestamp":1791928865504,"version":"4.3.4"},"reference-count":38,"publisher":"Association for Research in Vision and Ophthalmology (ARVO)","issue":"14","license":[{"start":{"date-parts":[[2025,11,14]],"date-time":"2025-11-14T00:00:00Z","timestamp":1763078400000},"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":[[2025,11,14]]},"DOI":"10.1167\/iovs.66.14.33","type":"journal-article","created":{"date-parts":[[2025,11,14]],"date-time":"2025-11-14T13:30:43Z","timestamp":1763127043000},"page":"33","source":"Crossref","is-referenced-by-count":3,"title":["Central Corneal Epithelial Wounding Accelerates Aniridia-Associated Keratopathy in PAX6-Deficient Mice"],"prefix":"10.1167","volume":"66","author":[{"given":"Yedizza","family":"Rautavaara","sequence":"first","affiliation":[{"name":"Division of Ophthalmology, Department of Biomedical and Clinical Sciences, Link\u00f6ping University, Link\u00f6ping, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Israa","family":"Sefawi","sequence":"additional","affiliation":[{"name":"Division of Ophthalmology, Department of Biomedical and Clinical Sciences, Link\u00f6ping University, Link\u00f6ping, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Meri","family":"Vattulainen","sequence":"additional","affiliation":[{"name":"Division of Ophthalmology, Department of Biomedical and Clinical Sciences, Link\u00f6ping University, Link\u00f6ping, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Petros","family":"Moustardas","sequence":"additional","affiliation":[{"name":"Division of Ophthalmology, Department of Biomedical and Clinical Sciences, Link\u00f6ping University, Link\u00f6ping, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dina","family":"Javidjam","sequence":"additional","affiliation":[{"name":"Division of Ophthalmology, Department of Biomedical and Clinical Sciences, Link\u00f6ping University, Link\u00f6ping, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ava","family":"Dashti","sequence":"additional","affiliation":[{"name":"Division of Ophthalmology, Department of Biomedical and Clinical Sciences, Link\u00f6ping University, Link\u00f6ping, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Neil","family":"Lagali","sequence":"additional","affiliation":[{"name":"Division of Ophthalmology, Department of Biomedical and Clinical Sciences, Link\u00f6ping University, Link\u00f6ping, Sweden"},{"name":"Department of Ophthalmology, S\u00f8rlandet Hospital, Arendal, Norway"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"27","reference":[{"key":"bib1","doi-asserted-by":"publisher","first-page":"621","DOI":"10.1016\/0039-6257(84)90184-X","article-title":"Aniridia. A review","volume":"28","author":"Nelson","year":"1984","journal-title":"Surv Ophthalmol"},{"key":"bib2","doi-asserted-by":"publisher","first-page":"1059","DOI":"10.1016\/0092-8674(91)90284-6","article-title":"Positional cloning and characterization of a paired box- and homeobox-containing gene from the aniridia region","volume":"67","author":"Ton","year":"1991","journal-title":"Cell"},{"key":"bib3","doi-asserted-by":"publisher","first-page":"1096","DOI":"10.1097\/ICO.0b013e3181d20493","article-title":"Corneal involvement in congenital aniridia","volume":"29","author":"Ed\u00e9n","year":"2010","journal-title":"Cornea"},{"key":"bib4","doi-asserted-by":"publisher","first-page":"479","DOI":"10.3341\/kjo.2014.28.6.479","article-title":"Congenital aniridia: long-term clinical course, visual outcome, and prognostic factors","volume":"28","author":"Chang","year":"2014","journal-title":"Korean J Ophthalmol"},{"key":"bib5","doi-asserted-by":"publisher","first-page":"235","DOI":"10.1186\/s12886-022-02460-5","article-title":"Clinical outcomes and visual prognostic factors in congenital aniridia","volume":"22","author":"Jacobson","year":"2022","journal-title":"BMC Ophthalmol"},{"key":"bib6","doi-asserted-by":"publisher","first-page":"130","DOI":"10.1016\/j.jtos.2019.11.002","article-title":"Early phenotypic features of aniridia-associated keratopathy and association with PAX6 coding mutations","volume":"18","author":"Lagali","year":"2020","journal-title":"Ocul Surf"},{"issue":"2","key":"bib7","doi-asserted-by":"publisher","first-page":"e183965","DOI":"10.1172\/jci.insight.183965","article-title":"A human-like model of aniridia-associated keratopathy for mechanistic and therapeutic studies","volume":"10","author":"Javidjam","journal-title":"JCI Insight"},{"key":"bib8","doi-asserted-by":"publisher","first-page":"273","DOI":"10.1016\/j.ophtha.2019.09.034","article-title":"PAX6 mutational status determines aniridia-associated keratopathy phenotype","volume":"127","author":"Lagali","year":"2020","journal-title":"Ophthalmology"},{"key":"bib9","doi-asserted-by":"publisher","first-page":"224","DOI":"10.1016\/j.exer.2005.02.002","article-title":"Developmental and cellular factors underlying corneal epithelial dysgenesis in the Pax6+\/- mouse model of aniridia","volume":"81","author":"Ramaesh","year":"2005","journal-title":"Exp Eye Res"},{"key":"bib10","doi-asserted-by":"publisher","first-page":"245","DOI":"10.1016\/j.jtos.2021.09.001","article-title":"Pathophysiology of aniridia-associated keratopathy: Developmental aspects and unanswered questions","volume":"22","author":"Latta","year":"2021","journal-title":"Ocular Surf"},{"key":"bib11","doi-asserted-by":"publisher","first-page":"421","DOI":"10.1002\/path.2371","article-title":"Chronic wound state exacerbated by oxidative stress in Pax6+\/- aniridia-related keratopathy","volume":"215","author":"Ou","year":"2008","journal-title":"J Pathol"},{"key":"bib12","doi-asserted-by":"publisher","first-page":"163","DOI":"10.1016\/j.jtos.2017.11.003","article-title":"Stage-related central corneal epithelial transformation in congenital aniridia-associated keratopathy","volume":"16","author":"Lagali","year":"2018","journal-title":"Ocul Surf"},{"key":"bib13","doi-asserted-by":"publisher","first-page":"30","DOI":"10.1136\/bjo-2021-320774","article-title":"Early ocular surface and tear film status in congenital aniridia indicates a supportive treatment window","volume":"108","author":"Fries","year":"2023","journal-title":"Br J Ophthalmol"},{"key":"bib14","doi-asserted-by":"publisher","first-page":"524","DOI":"10.1038\/s41434-018-0043-6","article-title":"Epistasis between Pax6Sey and genetic background reinforces the value of defined hybrid mouse models for therapeutic trials","volume":"25","author":"Hickmott","year":"2018","journal-title":"Gene Ther"},{"key":"bib15","doi-asserted-by":"crossref","first-page":"578","DOI":"10.1055\/a-2194-1580","article-title":"The effect of glaucoma treatment on aniridia-associated keratopathy (AAK)\u2014a report from the Homburg Register for Congenital Aniridia","volume":"242","author":"Fries","year":"2025","journal-title":"Klin Monbl Augenheilkd"},{"key":"bib16","doi-asserted-by":"publisher","first-page":"381","DOI":"10.1080\/02713683.2024.2438687","article-title":"The effect of lens properties on visual acuity, aniridia associated keratopathy and secondary glaucoma in congenital aniridia subjects","volume":"50","author":"N\u00e1ray","year":"2025","journal-title":"Curr Eye Res"},{"key":"bib17","doi-asserted-by":"publisher","first-page":"927","DOI":"10.1016\/j.exer.2011.10.012","article-title":"Removal of the basement membrane enhances corneal wound healing","volume":"93","author":"Pal-Ghosh","year":"2011","journal-title":"Exp Eye Res"},{"key":"bib18","doi-asserted-by":"publisher","first-page":"671","DOI":"10.1007\/s40123-023-00880-0","article-title":"Animal models for limbal stem cell deficiency: a critical narrative literature review","volume":"13","author":"Atalay","year":"2024","journal-title":"Ophthalmol Ther"},{"key":"bib19","doi-asserted-by":"publisher","first-page":"78","DOI":"10.1016\/j.jprot.2013.09.003","article-title":"Analysis of protein composition and protein expression in the tear fluid of patients with congenital aniridia","volume":"94","author":"Ihnatko","year":"2013","journal-title":"J Proteomics"},{"issue":"1","key":"bib20","doi-asserted-by":"publisher","first-page":"9763","DOI":"10.1038\/s41598-019-45100-7","article-title":"Pax 6 controls neural crest potential of limbal niche cells to support self-renewal of limbal epithelial stem cells","volume":"9","author":"Chen","year":"2019","journal-title":"Sci Rep"},{"issue":"8","key":"bib21","doi-asserted-by":"publisher","first-page":"e71117","DOI":"10.1371\/journal.pone.0071117","article-title":"Increased corneal epithelial turnover contributes to abnormal homeostasis in the Pax6+\/- mouse model of aniridia","volume":"8","author":"Douvaras","year":"2013","journal-title":"PLoS One"},{"key":"bib22","doi-asserted-by":"publisher","first-page":"5276","DOI":"10.1167\/iovs.06-0581","article-title":"Cell surface glycoconjugate abnormalities and corneal epithelial wound healing in the Pax6+\/- mouse model of aniridia-related keratopathy","volume":"47","author":"Kucerova","year":"2006","journal-title":"Invest Ophthalmol Vis Sci"},{"key":"bib23","doi-asserted-by":"publisher","first-page":"47","DOI":"10.1111\/j.1755-3768.2009.01603.x","article-title":"Immunohistochemical expression of epithelial cell markers in corneas with congenital aniridia and ocular cicatrizing pemphigoid","volume":"89","author":"Auw-Haedrich","year":"2011","journal-title":"Acta Ophthalmol"},{"key":"bib24","doi-asserted-by":"publisher","first-page":"447","DOI":"10.1016\/j.jtos.2019.05.005","article-title":"Insulin-like growth factor binding protein-7: a marker of conjunctivalization in an animal model of limbal stem cell deficiency","volume":"17","author":"Park","year":"2019","journal-title":"Ocul Surf"},{"key":"bib25","doi-asserted-by":"publisher","first-page":"2032","DOI":"10.1002\/stem.1176","article-title":"Corneal goblet cells and their niche: implications for corneal stem cell deficiency","volume":"30","author":"Pajoohesh-Ganji","year":"2012","journal-title":"Stem Cells"},{"key":"bib26","doi-asserted-by":"publisher","first-page":"1535","DOI":"10.1016\/j.ophtha.2005.04.014","article-title":"Association of aniridia and dry eyes","volume":"112","author":"Jastaneiah","year":"2005","journal-title":"Ophthalmology"},{"key":"bib27","doi-asserted-by":"publisher","first-page":"107915","DOI":"10.1016\/j.exer.2020.107915","article-title":"Dynamic spatiotemporal expression pattern of limbal stem cell putative biomarkers during mouse development","volume":"192","author":"Guo","year":"2020","journal-title":"Exp Eye Res"},{"key":"bib28","first-page":"323","article-title":"The spectrum of cytokeratins expressed in the adult human cornea, limbus and perilimbal conjunctiva","volume":"26","author":"Merjava","year":"2011","journal-title":"Histol Histopathol"},{"issue":"1","key":"bib29","doi-asserted-by":"publisher","first-page":"10268","DOI":"10.1038\/s41598-018-28596-3","article-title":"Transcription factor profiling identifies Sox9 as regulator of proliferation and differentiation in corneal epithelial stem\/progenitor cells","volume":"8","author":"Menzel-Severing","year":"2018","journal-title":"Sci Rep"},{"key":"bib30","article-title":"Sox9 prevents retinal degeneration and is required for limbal stem cell differentiation in the adult mouse eye","volume":"13","author":"Hurtado","year":"2024","journal-title":"eLife"},{"issue":"10","key":"bib32","doi-asserted-by":"publisher","first-page":"e30840","DOI":"10.1016\/j.heliyon.2024.e30840","article-title":"Role of macrophage in ocular neovascularization","volume":"10","author":"Tu","year":"2024","journal-title":"Heliyon"},{"key":"bib33","first-page":"2905","article-title":"The inflammatory milieu associated with conjunctivalized cornea and its alteration with IL-1 RA gene therapy","volume":"43","author":"Moore","year":"2002","journal-title":"Invest Ophthalmol Vis Sci"},{"key":"bib34","doi-asserted-by":"publisher","first-page":"152","DOI":"10.1016\/j.exer.2009.03.001","article-title":"Corneal myofibroblast viability: opposing effects of IL-1 and TGF beta1","volume":"89","author":"Kaur","year":"2009","journal-title":"Exp Eye Res"},{"key":"bib35","doi-asserted-by":"publisher","first-page":"456","DOI":"10.1016\/j.exer.2010.06.023","article-title":"Stromal interleukin-1 expression in the cornea after haze-associated injury","volume":"91","author":"Barbosa","year":"2010","journal-title":"Exp Eye Res"},{"issue":"4","key":"bib36","doi-asserted-by":"publisher","first-page":"8","DOI":"10.1167\/iovs.62.4.8","article-title":"Interleukin-1 and transforming growth factor beta: commonly opposing, but sometimes supporting, master regulators of the corneal wound healing response to injury","volume":"62","author":"Wilson","year":"2021","journal-title":"Invest Ophthalmol Vis Sci"},{"key":"bib37","doi-asserted-by":"publisher","first-page":"166","DOI":"10.1016\/j.exer.2009.03.003","article-title":"Corneal epithelial proliferation and thickness in a mouse model of dry eye","volume":"89","author":"Fabiani","year":"2009","journal-title":"Exp Eye Res"},{"issue":"12","key":"bib38","doi-asserted-by":"publisher","first-page":"115","DOI":"10.3390\/jcm6120115","article-title":"Adipose derived stem cells for corneal wound healing after laser induced corneal lesions in mice","volume":"6","author":"Zeppieri","year":"2017","journal-title":"J Clin Med"},{"key":"bib31","article-title":"Sox9 marks limbal stem cells and is required for asymmetric cell fate switch in the corneal epithelium","author":"Rice","year":"2024"}],"container-title":["Investigative Ophthalmology & Visual Science"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/iovs.arvojournals.org\/article.aspx?articleid=2811092","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T21:32:03Z","timestamp":1791927123000},"score":1,"resource":{"primary":{"URL":"https:\/\/iovs.arvojournals.org\/article.aspx?articleid=2811092"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,11,14]]},"references-count":38,"journal-issue":{"issue":"14","published-print":{"date-parts":[[2025,11,3]]}},"URL":"https:\/\/doi.org\/10.1167\/iovs.66.14.33","relation":{},"ISSN":["1552-5783"],"issn-type":[{"value":"1552-5783","type":"print"}],"subject":[],"published":{"date-parts":[[2025,11,14]]}}}