{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,4]],"date-time":"2026-11-04T12:40:09Z","timestamp":1793796009381,"version":"4.6.1"},"reference-count":122,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2024,11,1]],"date-time":"2024-11-01T00:00:00Z","timestamp":1730419200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2024,11,1]],"date-time":"2024-11-01T00:00:00Z","timestamp":1730419200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2024,9,16]],"date-time":"2024-09-16T00:00:00Z","timestamp":1726444800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Progress in Retinal and Eye Research"],"published-print":{"date-parts":[[2024,11]]},"DOI":"10.1016\/j.preteyeres.2024.101299","type":"journal-article","created":{"date-parts":[[2024,9,6]],"date-time":"2024-09-06T21:27:26Z","timestamp":1725658046000},"page":"101299","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":14,"special_numbering":"C","title":["The role of CFTR in the eye, and the effect of early highly effective modulator treatment for cystic fibrosis on eye health"],"prefix":"10.1016","volume":"103","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6044-4824","authenticated-orcid":false,"given":"Elena K.","family":"Schneider-Futschik","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yimin","family":"Zhu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Danni","family":"Li","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mark D.","family":"Habgood","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bao N.","family":"Nguyen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ines","family":"Pankonien","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Margarida D.","family":"Amaral","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura E.","family":"Downie","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Holly R.","family":"Chinnery","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"issue":"2","key":"10.1016\/j.preteyeres.2024.101299_bib1","doi-asserted-by":"crossref","first-page":"113","DOI":"10.1016\/j.sjopt.2013.01.001","article-title":"Lacrimal glands in cystic fibrosis","volume":"27","author":"Alghadyan","year":"2013","journal-title":"Saudi J Ophthalmol"},{"issue":"Suppl. 1","key":"10.1016\/j.preteyeres.2024.101299_bib2","doi-asserted-by":"crossref","first-page":"S33","DOI":"10.1016\/j.jcf.2019.10.017","article-title":"CFTR processing, trafficking and interactions","volume":"19","author":"Amaral","year":"2020","journal-title":"J. Cyst. Fibros."},{"issue":"7","key":"10.1016\/j.preteyeres.2024.101299_bib3","doi-asserted-by":"crossref","first-page":"334","DOI":"10.1016\/j.tips.2007.05.004","article-title":"Molecular targeting of CFTR as a therapeutic approach to cystic fibrosis","volume":"28","author":"Amaral","year":"2007","journal-title":"Trends Pharmacol. Sci."},{"issue":"9","key":"10.1016\/j.preteyeres.2024.101299_bib4","doi-asserted-by":"crossref","DOI":"10.3390\/ijms21093133","article-title":"What role does CFTR play in development, differentiation, regeneration and cancer?","volume":"21","author":"Amaral","year":"2020","journal-title":"Int. J. Mol. Sci."},{"issue":"6","key":"10.1016\/j.preteyeres.2024.101299_bib5","doi-asserted-by":"crossref","first-page":"688","DOI":"10.1136\/bjo.83.6.688","article-title":"Ocular signs and symptoms and vitamin A status in patients with cystic fibrosis treated with daily vitamin A supplements","volume":"83","author":"Ansari","year":"1999","journal-title":"Br. J. Ophthalmol."},{"key":"10.1016\/j.preteyeres.2024.101299_bib6","article-title":"Congenital cataract: a guide to genetic and clinical management","volume":"1","author":"Bell","year":"2020","journal-title":"Ther. Adv. Respir. Dis."},{"key":"10.1016\/j.preteyeres.2024.101299_bib8","doi-asserted-by":"crossref","first-page":"30","DOI":"10.1016\/j.spen.2019.05.006","article-title":"The assessment of visual function and functional vision","volume":"31","author":"Bennett","year":"2019","journal-title":"Semin. Pediatr. Neurol."},{"issue":"1","key":"10.1016\/j.preteyeres.2024.101299_bib9","doi-asserted-by":"crossref","first-page":"54","DOI":"10.1167\/iovs.17-22533","article-title":"Novel insight into the role of CFTR in lacrimal gland duct function in mice","volume":"59","author":"Berczeli","year":"2018","journal-title":"Invest. Ophthalmol. Vis. Sci."},{"issue":"2","key":"10.1016\/j.preteyeres.2024.101299_bib10","first-page":"103","article-title":"Tear Lysozyme in Sjogren s syndrome, Meibomian gland dysfunction, and non-dry-eye","volume":"85","author":"Berra","year":"2021","journal-title":"Arq. Bras. Oftalmol."},{"issue":"1","key":"10.1016\/j.preteyeres.2024.101299_bib11","doi-asserted-by":"crossref","first-page":"43","DOI":"10.1023\/A:1022514031645","article-title":"Retinal pigment epithelial function: a role for CFTR?","volume":"106","author":"Blaug","year":"2003","journal-title":"Doc. Ophthalmol."},{"issue":"3","key":"10.1016\/j.preteyeres.2024.101299_bib12","doi-asserted-by":"crossref","first-page":"354","DOI":"10.1001\/archopht.1990.01070050052029","article-title":"Xerophthalmia and cystic fibrosis","volume":"108","author":"Brooks","year":"1990","journal-title":"Arch. Ophthalmol."},{"issue":"6","key":"10.1016\/j.preteyeres.2024.101299_bib13","doi-asserted-by":"crossref","first-page":"693","DOI":"10.1038\/aps.2011.71","article-title":"Targeting F508del-CFTR to develop rational new therapies for cystic fibrosis","volume":"32","author":"Cai","year":"2011","journal-title":"Acta Pharmacol. Sin."},{"issue":"1","key":"10.1016\/j.preteyeres.2024.101299_bib14","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1177\/112067210101100103","article-title":"The eye in cystic fibrosis","volume":"11","author":"Castagna","year":"2001","journal-title":"Eur. J. Ophthalmol."},{"issue":"1","key":"10.1016\/j.preteyeres.2024.101299_bib15","doi-asserted-by":"crossref","first-page":"600","DOI":"10.1038\/s41598-022-24429-6","article-title":"Lipidomic alterations in human saliva from cystic fibrosis patients","volume":"13","author":"Caterino","year":"2023","journal-title":"Sci. Rep."},{"key":"10.1016\/j.preteyeres.2024.101299_bib16","article-title":"Comparison of two pregnancies with and without elexacaftor-tezacaftor-ivacaftor in a woman with cystic fibrosis","volume":"83","author":"Chamagne","year":"2022","journal-title":"Respir Med Res"},{"issue":"3","key":"10.1016\/j.preteyeres.2024.101299_bib17","doi-asserted-by":"crossref","first-page":"147","DOI":"10.1089\/jop.2019.0087","article-title":"Nanomolar potency aminophenyltriazine CFTR activator reverses corneal epithelial injury in a mouse model of dry eye","volume":"36","author":"Chen","year":"2020","journal-title":"J. Ocul. Pharmacol. Therapeut."},{"issue":"4","key":"10.1016\/j.preteyeres.2024.101299_bib18","doi-asserted-by":"crossref","first-page":"509","DOI":"10.1038\/nm.2715","article-title":"Cystic fibrosis: a mucosal immunodeficiency syndrome","volume":"18","author":"Cohen","year":"2012","journal-title":"Nat Med"},{"issue":"12","key":"10.1016\/j.preteyeres.2024.101299_bib19","doi-asserted-by":"crossref","first-page":"L917","DOI":"10.1152\/ajplung.00326.2014","article-title":"CFTR and lung homeostasis","volume":"307","author":"Collawn","year":"2014","journal-title":"Am. J. Physiol. Lung Cell Mol. Physiol."},{"issue":"3","key":"10.1016\/j.preteyeres.2024.101299_bib20","doi-asserted-by":"crossref","first-page":"276","DOI":"10.1016\/j.jtos.2017.05.008","article-title":"TFOS DEWS II definition and classification report","volume":"15","author":"Craig","year":"2017","journal-title":"Ocul. Surf."},{"issue":"8","key":"10.1016\/j.preteyeres.2024.101299_bib21","doi-asserted-by":"crossref","first-page":"662","DOI":"10.1016\/S2213-2600(16)00023-0","article-title":"Progress in therapies for cystic fibrosis","volume":"4","author":"De Boeck","year":"2016","journal-title":"Lancet Respir. Med."},{"issue":"6","key":"10.1016\/j.preteyeres.2024.101299_bib22","doi-asserted-by":"crossref","first-page":"2960","DOI":"10.1167\/iovs.09-4687","article-title":"Duct system of the rabbit lacrimal gland: structural characteristics and role in lacrimal secretion","volume":"51","author":"Ding","year":"2010","journal-title":"Invest. Ophthalmol. Vis. Sci."},{"issue":"2","key":"10.1016\/j.preteyeres.2024.101299_bib23","doi-asserted-by":"crossref","first-page":"132","DOI":"10.1016\/j.clae.2021.02.009","article-title":"Clear - anatomy and physiology of the anterior eye","volume":"44","author":"Downie","year":"2021","journal-title":"Contact Lens Anterior Eye"},{"issue":"31","key":"10.1016\/j.preteyeres.2024.101299_bib24","doi-asserted-by":"crossref","DOI":"10.1073\/pnas.2217795120","article-title":"Redefining the human corneal immune compartment using dynamic intravital imaging","volume":"120","author":"Downie","year":"2023","journal-title":"Proc. Natl. Acad. Sci. U.S.A."},{"key":"10.1016\/j.preteyeres.2024.101299_bib25","series-title":"Symkevi","year":"2020"},{"issue":"6","key":"10.1016\/j.preteyeres.2024.101299_bib26","doi-asserted-by":"crossref","first-page":"527","DOI":"10.1080\/13816810.2017.1301964","article-title":"Diagnosed cataracts in patients with cystic fibrosis in a United States administrative database","volume":"38","author":"Everage","year":"2017","journal-title":"Ophthalmic Genet."},{"issue":"3","key":"10.1016\/j.preteyeres.2024.101299_bib27","doi-asserted-by":"crossref","first-page":"178","DOI":"10.1159\/000027272","article-title":"Cystic fibrosis and lens opacity","volume":"212","author":"Fam\u00e0","year":"1998","journal-title":"Ophthalmologica"},{"key":"10.1016\/j.preteyeres.2024.101299_bib28","article-title":"Ivacaftor-lumacaftor","volume":"2018","year":"2018","journal-title":"United States Food and Drug Administration"},{"key":"10.1016\/j.preteyeres.2024.101299_bib29","article-title":"Ivacaftor-tezacaftor","volume":"2020","year":"2020","journal-title":"United States Food and Drug Administration"},{"key":"10.1016\/j.preteyeres.2024.101299_bib30","article-title":"Ivacaftor","volume":"2020","year":"2020","journal-title":"United States Food and Drug Administration"},{"key":"10.1016\/j.preteyeres.2024.101299_bib31","article-title":"Trikafta (elexacaftor-tezacaftor-ivacaftor)","volume":"2021","year":"2021","journal-title":"United States Food and Drug Administration"},{"issue":"5","key":"10.1016\/j.preteyeres.2024.101299_bib34","doi-asserted-by":"crossref","first-page":"1789","DOI":"10.1096\/fj.201500180","article-title":"Small-molecule CFTR activators increase tear secretion and prevent experimental dry eye disease","volume":"30","author":"Flores","year":"2016","journal-title":"Faseb. J."},{"issue":"5","key":"10.1016\/j.preteyeres.2024.101299_bib35","doi-asserted-by":"crossref","first-page":"835","DOI":"10.1016\/j.jcf.2021.03.018","article-title":"Normal pancreatic function and false-negative CF newborn screen in a child born to a mother taking CFTR modulator therapy during pregnancy","volume":"20","author":"Fortner","year":"2021","journal-title":"J. Cyst. Fibros."},{"key":"10.1016\/j.preteyeres.2024.101299_bib36","article-title":"Tear osmolarity in pediatric patients with cystic fibrosis","author":"Franco","year":"2013","journal-title":"ARVO Annual Meeting"},{"issue":"1","key":"10.1016\/j.preteyeres.2024.101299_bib125","doi-asserted-by":"crossref","first-page":"376","DOI":"10.1038\/s41392-022-01191-9","article-title":"The Hippo signalling pathway and its implications in human health and diseases","volume":"7","author":"Fu","year":"2022","journal-title":"Signal Transduct Target Ther"},{"issue":"2","key":"10.1016\/j.preteyeres.2024.101299_bib37","doi-asserted-by":"crossref","first-page":"383","DOI":"10.1016\/j.chest.2018.04.036","article-title":"Ion Channel modulators in cystic fibrosis","volume":"154","author":"Gentzsch","year":"2018","journal-title":"Chest"},{"issue":"1","key":"10.1016\/j.preteyeres.2024.101299_bib38","doi-asserted-by":"crossref","first-page":"4","DOI":"10.1021\/acsptsci.9b00060","article-title":"Emerging cystic fibrosis transmembrane conductance regulator modulators as new drugs for cystic fibrosis: a portrait of in vitro pharmacology and clinical translation","volume":"3","author":"Ghelani","year":"2020","journal-title":"ACS Pharmacol. Transl. Sci."},{"key":"10.1016\/j.preteyeres.2024.101299_bib39","article-title":"Ophthalmologic manifestations of adult patients with cystic fibrosis","author":"Giannakouras","year":"2021","journal-title":"Eur. J. Ophthalmol."},{"key":"10.1016\/j.preteyeres.2024.101299_bib40","doi-asserted-by":"crossref","first-page":"157","DOI":"10.1016\/bs.apcsb.2015.11.006","article-title":"Ion channels in the eye: involvement in ocular pathologies","volume":"104","author":"Giblin","year":"2016","journal-title":"Adv Protein Chem Struct Biol"},{"key":"10.1016\/j.preteyeres.2024.101299_bib41","doi-asserted-by":"crossref","DOI":"10.1164\/rccm.202301-0084OC","article-title":"Phase 3 Open-Label Clinical Trial of Elexacaftor\/Tezacaftor\/Ivacaftor in Children Aged 2 Through 5 Years with Cystic Fibrosis and at Least One F508del Allele","author":"Goralski","year":"2023","journal-title":"Am. J. Respir. Crit. Care Med."},{"issue":"Online","key":"10.1016\/j.preteyeres.2024.101299_bib42","first-page":"e56","article-title":"Diffuse retinal pigment epithelial disease in an adult with cystic fibrosis","volume":"42","author":"Goren","year":"2011","journal-title":"Ophthalmic Surg. Laser. Imag."},{"key":"10.1016\/j.preteyeres.2024.101299_bib43","doi-asserted-by":"crossref","first-page":"105","DOI":"10.1007\/978-1-4614-3209-8_14","article-title":"Rescue of compromised lysosomes enhances degradation of photoreceptor outer segments and reduces lipofuscin-like autofluorescence in retinal pigmented epithelial cells","volume":"801","author":"Guha","year":"2014","journal-title":"Adv. Exp. Med. Biol."},{"issue":"3","key":"10.1016\/j.preteyeres.2024.101299_bib44","doi-asserted-by":"crossref","first-page":"456","DOI":"10.1016\/j.jcf.2022.01.009","article-title":"Worldwide rates of diagnosis and effective treatment for cystic fibrosis","volume":"21","author":"Guo","year":"2022","journal-title":"J. Cyst. Fibros."},{"issue":"1","key":"10.1016\/j.preteyeres.2024.101299_bib45","doi-asserted-by":"crossref","first-page":"15","DOI":"10.1186\/s13167-016-0065-3","article-title":"Tear fluid biomarkers in ocular and systemic disease: potential use for predictive, preventive and personalised medicine","volume":"7","author":"Hagan","year":"2016","journal-title":"EPMA J."},{"issue":"1_Suppl. l","key":"10.1016\/j.preteyeres.2024.101299_bib46","doi-asserted-by":"crossref","first-page":"30","DOI":"10.1258\/jrsm.2010.s11008","article-title":"Cystic fibrosis presenting with corneal perforation and crystalline lens extrusion","volume":"103","author":"Hall","year":"2010","journal-title":"J. R. Soc. Med."},{"issue":"5","key":"10.1016\/j.preteyeres.2024.101299_bib47","doi-asserted-by":"crossref","first-page":"C1332","DOI":"10.1152\/ajpcell.1998.274.5.C1332","article-title":"Aquaporins in complex tissues: distribution of aquaporins 1-5 in human and rat eye","volume":"274","author":"Hamann","year":"1998","journal-title":"Am. J. Physiol."},{"issue":"2","key":"10.1016\/j.preteyeres.2024.101299_bib48","doi-asserted-by":"crossref","first-page":"167","DOI":"10.1111\/j.1755-3768.1975.tb01151.x","article-title":"Conjunctival goblet cells in patients with cystic fibrosis","volume":"53","author":"Holm","year":"1975","journal-title":"Acta Ophthalmol."},{"key":"10.1016\/j.preteyeres.2024.101299_bib49","author":"Incorporated"},{"key":"10.1016\/j.preteyeres.2024.101299_bib50","author":"Incorporated"},{"issue":"6","key":"10.1016\/j.preteyeres.2024.101299_bib52","doi-asserted-by":"crossref","first-page":"1074","DOI":"10.1016\/j.jcf.2022.10.004","article-title":"Congenital bilateral cataracts in newborns exposed to elexacaftor-tezacaftor-ivacaftor in utero and while breast feeding","volume":"21","author":"Jain","year":"2022","journal-title":"J. Cyst. Fibros."},{"issue":"5","key":"10.1016\/j.preteyeres.2024.101299_bib53","doi-asserted-by":"crossref","first-page":"575","DOI":"10.1038\/sj.eye.6701582","article-title":"Retinal nerve fibre layer measurements are reduced in patients with obstructive sleep apnoea syndrome","volume":"19","author":"Kargi","year":"2005","journal-title":"Eye"},{"issue":"5","key":"10.1016\/j.preteyeres.2024.101299_bib54","doi-asserted-by":"crossref","first-page":"2842","DOI":"10.1152\/jn.00511.2017","article-title":"A role for the cystic fibrosis transmembrane conductance regulator in the nitric oxide-dependent release of Cl(-) from acidic organelles in amacrine cells","volume":"118","author":"Krishnan","year":"2017","journal-title":"J. Neurophysiol."},{"issue":"1","key":"10.1016\/j.preteyeres.2024.101299_bib55","doi-asserted-by":"crossref","DOI":"10.1080\/19336950.2023.2186434","article-title":"SLC26A9 in airways and intestine: secretion or absorption?","volume":"17","author":"Kunzelmann","year":"2023","journal-title":"Channels"},{"issue":"7","key":"10.1016\/j.preteyeres.2024.101299_bib56","doi-asserted-by":"crossref","first-page":"2641","DOI":"10.4103\/ijo.IJO_2927_21","article-title":"Bilateral Pseudomonas aeruginosa keratitis as presenting feature in an infant of cystic fibrosis","volume":"70","author":"Lakshmipathy","year":"2022","journal-title":"Indian J. Ophthalmol."},{"issue":"6","key":"10.1016\/j.preteyeres.2024.101299_bib124","doi-asserted-by":"crossref","first-page":"794","DOI":"10.1002\/dvdy.24628","article-title":"Hippo-yap signaling in ocular development and disease","volume":"247","author":"Lee","year":"2018","journal-title":"Dev Dyn"},{"issue":"2","key":"10.1016\/j.preteyeres.2024.101299_bib57","doi-asserted-by":"crossref","first-page":"105","DOI":"10.1007\/s00232-005-0849-1","article-title":"Aquaporins and CFTR in ocular epithelial fluid transport","volume":"210","author":"Levin","year":"2006","journal-title":"J. Membr. Biol."},{"issue":"2","key":"10.1016\/j.preteyeres.2024.101299_bib58","doi-asserted-by":"crossref","first-page":"126","DOI":"10.1016\/j.oret.2023.08.021","article-title":"Characteristics of vitamin A deficiency retinopathy at a tertiary referral center in the United States","volume":"8","author":"Levine","year":"2024","journal-title":"Ophthalmol Retina"},{"issue":"3","key":"10.1016\/j.preteyeres.2024.101299_bib59","doi-asserted-by":"crossref","first-page":"355","DOI":"10.1021\/acsptsci.2c00233","article-title":"In utero mapping and development role of CFTR in lung and gastrointestinal tract of cystic fibrosis patients","volume":"6","author":"Li","year":"2023","journal-title":"ACS Pharmacol. Transl. Sci."},{"issue":"3","key":"10.1016\/j.preteyeres.2024.101299_bib60","article-title":"Current and emerging inhaled antibiotics for chronic pulmonary Pseudomonas aeruginosa and Staphylococcus aureus infections in cystic fibrosis","volume":"12","author":"Li","year":"2023","journal-title":"Antibiotics (Basel)"},{"key":"10.1016\/j.preteyeres.2024.101299_bib61","doi-asserted-by":"crossref","DOI":"10.1016\/j.biopha.2024.116155","article-title":"Fetal drug exposure after maternally administered CFTR modulators Elexacaftor\/Tezacaftor\/Ivacaftor in a rat model","volume":"171","author":"Li","year":"2024","journal-title":"Biomed. Pharmacother."},{"issue":"12","key":"10.1016\/j.preteyeres.2024.101299_bib62","doi-asserted-by":"crossref","DOI":"10.3390\/ijms25126692","article-title":"Ocular changes in cystic fibrosis: a review","volume":"25","author":"Liberski","year":"2024","journal-title":"Int. J. Mol. Sci."},{"issue":"2","key":"10.1016\/j.preteyeres.2024.101299_bib63","doi-asserted-by":"crossref","first-page":"C160","DOI":"10.1152\/ajpcell.00278.2011","article-title":"Cystic fibrosis transmembrane conductance regulator contributes to reacidification of alkalinized lysosomes in RPE cells","volume":"303","author":"Liu","year":"2012","journal-title":"Am. J. Physiol. Cell Physiol."},{"issue":"8","key":"10.1016\/j.preteyeres.2024.101299_bib64","doi-asserted-by":"crossref","first-page":"671","DOI":"10.3109\/02713683.2012.675613","article-title":"CFTR-mediated Cl(-) transport in the acinar and duct cells of rabbit lacrimal gland","volume":"37","author":"Lu","year":"2012","journal-title":"Curr. Eye Res."},{"issue":"1","key":"10.1016\/j.preteyeres.2024.101299_bib65","first-page":"160","article-title":"A study of aqueous humor formation in patients with cystic fibrosis","volume":"33","author":"McCannel","year":"1992","journal-title":"Invest. Ophthalmol. Vis. Sci."},{"issue":"S1","key":"10.1016\/j.preteyeres.2024.101299_bib66","doi-asserted-by":"crossref","first-page":"S57","DOI":"10.1002\/ncp.11122","article-title":"Cystic fibrosis and fat malabsorption: pathophysiology of the cystic fibrosis gastrointestinal tract and the impact of highly effective CFTR modulator therapy","volume":"39","author":"McDonald","year":"2024","journal-title":"Nutr. Clin. Pract."},{"issue":"8","key":"10.1016\/j.preteyeres.2024.101299_bib67","doi-asserted-by":"crossref","first-page":"1196","DOI":"10.1016\/j.ophtha.2019.03.014","article-title":"Tear film extensional viscosity is a novel potential biomarker of dry eye disease","volume":"126","author":"McDonnell","year":"2019","journal-title":"Ophthalmology"},{"issue":"5","key":"10.1016\/j.preteyeres.2024.101299_bib68","doi-asserted-by":"crossref","first-page":"316","DOI":"10.1136\/bjo.68.5.316","article-title":"Normal tear protein profiles and age-related changes","volume":"68","author":"McGill","year":"1984","journal-title":"Br. J. Ophthalmol."},{"issue":"10","key":"10.1016\/j.preteyeres.2024.101299_bib69","doi-asserted-by":"crossref","first-page":"1359","DOI":"10.1515\/hsz-2018-0470","article-title":"CFTR structure, stability, function and regulation","volume":"400","author":"Meng","year":"2019","journal-title":"Biol. Chem."},{"issue":"5","key":"10.1016\/j.preteyeres.2024.101299_bib70","first-page":"71","article-title":"The pathophysiology, diagnosis, and treatment of dry eye disease","volume":"112","author":"Messmer","year":"2015","journal-title":"Dtsch Arztebl Int"},{"issue":"19","key":"10.1016\/j.preteyeres.2024.101299_bib71","doi-asserted-by":"crossref","first-page":"1809","DOI":"10.1056\/NEJMoa1908639","article-title":"Elexacaftor-Tezacaftor-Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele","volume":"381","author":"Middleton","year":"2019","journal-title":"N. Engl. J. Med."},{"issue":"12","key":"10.1016\/j.preteyeres.2024.101299_bib72","doi-asserted-by":"crossref","first-page":"7174","DOI":"10.1073\/pnas.95.12.7174","article-title":"A release mechanism for stored ATP in ocular ciliary epithelial cells","volume":"95","author":"Mitchell","year":"1998","journal-title":"Proc. Natl. Acad. Sci. U.S.A."},{"key":"10.1016\/j.preteyeres.2024.101299_bib73","first-page":"647","article-title":"Corneal refractive surgery considerations in patients with cystic fibrosis and cystic fibrosis transmembrane conductance regulator-related disorders","volume":"15","author":"Moshirfar","year":"2022","journal-title":"Int. Med. Case Rep. J."},{"issue":"1\u20133","key":"10.1016\/j.preteyeres.2024.101299_bib74","first-page":"90","article-title":"[Evaluation of the tears secretion in young patients with cystic fibrosis]","volume":"107","author":"Mrugacz","year":"2005","journal-title":"Klin. Oczna."},{"issue":"2","key":"10.1016\/j.preteyeres.2024.101299_bib75","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1089\/jir.2006.26.71","article-title":"IL-8 and IFN-gamma in tear fluid of patients with cystic fibrosis","volume":"26","author":"Mrugacz","year":"2006","journal-title":"J. Interferon Cytokine Res."},{"issue":"9","key":"10.1016\/j.preteyeres.2024.101299_bib76","doi-asserted-by":"crossref","first-page":"1137","DOI":"10.1038\/sj.eye.6702867","article-title":"Impression cytology of the conjunctival epithelial cells in patients with cystic fibrosis","volume":"22","author":"Mrugacz","year":"2008","journal-title":"Eye"},{"issue":"4","key":"10.1016\/j.preteyeres.2024.101299_bib77","doi-asserted-by":"crossref","first-page":"202","DOI":"10.1007\/s00431-004-1587-6","article-title":"Retinol binding protein status in relation to ocular surface changes in patients with cystic fibrosis treated with daily vitamin A supplements","volume":"164","author":"Mrugacz","year":"2005","journal-title":"Eur. J. Pediatr."},{"issue":"3","key":"10.1016\/j.preteyeres.2024.101299_bib78","doi-asserted-by":"crossref","first-page":"204","DOI":"10.1002\/cyto.b.20159","article-title":"ICAM-1 expression on conjunctival epithelial cells in patients with cystic fibrosis","volume":"72","author":"Mrugacz","year":"2007","journal-title":"Cytometry B Clin Cytom"},{"issue":"8","key":"10.1016\/j.preteyeres.2024.101299_bib79","doi-asserted-by":"crossref","first-page":"1062","DOI":"10.1038\/sj.eye.6702435","article-title":"Flow cytometric analysis of HLA-DR antigen in conjunctival epithelial cells of patients with cystic fibrosis","volume":"21","author":"Mrugacz","year":"2007","journal-title":"Eye"},{"issue":"4\u20136","key":"10.1016\/j.preteyeres.2024.101299_bib80","first-page":"184","article-title":"[Concentration of interleukine-8 in tears in patients with cystic fibrosis]","volume":"108","author":"Mrugacz","year":"2006","journal-title":"Klin. Oczna."},{"issue":"1","key":"10.1016\/j.preteyeres.2024.101299_bib81","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1001\/archopht.124.1.79","article-title":"Development of cataract and associated risk factors: the visual impairment Project","volume":"124","author":"Mukesh","year":"2006","journal-title":"Arch. Ophthalmol."},{"issue":"1","key":"10.1016\/j.preteyeres.2024.101299_bib82","doi-asserted-by":"crossref","DOI":"10.1242\/dev.155838","article-title":"Light-focusing human micro-lenses generated from pluripotent stem cells model lens development and drug-induced cataract in vitro","volume":"145","author":"Murphy","year":"2018","journal-title":"Development"},{"issue":"4","key":"10.1016\/j.preteyeres.2024.101299_bib83","doi-asserted-by":"crossref","first-page":"521","DOI":"10.1016\/j.jcf.2020.02.018","article-title":"Outcomes of pregnancy in women with cystic fibrosis (CF) taking CFTR modulators - an international survey","volume":"19","author":"Nash","year":"2020","journal-title":"J. Cyst. Fibros."},{"key":"10.1016\/j.preteyeres.2024.101299_bib84","doi-asserted-by":"crossref","DOI":"10.1155\/2014\/580373","article-title":"Cystic fibrosis and new trends by ophthalmological evaluation: a pilot study","volume":"2014","author":"Nebbioso","year":"2014","journal-title":"BioMed Res. Int."},{"issue":"5","key":"10.1016\/j.preteyeres.2024.101299_bib85","doi-asserted-by":"crossref","DOI":"10.3390\/ijms23052688","article-title":"CFTR, cell junctions and the cytoskeleton","volume":"23","author":"Pankonien","year":"2022","journal-title":"Int. J. Mol. Sci."},{"issue":"11","key":"10.1016\/j.preteyeres.2024.101299_bib86","doi-asserted-by":"crossref","first-page":"20","DOI":"10.1167\/tvst.9.11.20","article-title":"Ocular surface potential difference measured in human subjects to study ocular surface ion transport","volume":"9","author":"Pasricha","year":"2020","journal-title":"Transl Vis Sci Technol"},{"issue":"3","key":"10.1016\/j.preteyeres.2024.101299_bib87","doi-asserted-by":"crossref","first-page":"307","DOI":"10.1097\/QAI.0b013e31828ad759","article-title":"Increased ocular lens density in HIV-infected individuals with low nadir CD4 counts in South Africa: evidence of accelerated aging","volume":"63","author":"Pathai","year":"2013","journal-title":"J. Acquir. Immune Defic. Syndr."},{"issue":"7","key":"10.1016\/j.preteyeres.2024.101299_bib88","doi-asserted-by":"crossref","DOI":"10.1016\/j.heliyon.2023.e17553","article-title":"Role of innate immunity and systemic inflammation in cystic fibrosis disease progression","volume":"9","author":"Purushothaman","year":"2023","journal-title":"Heliyon"},{"issue":"5","key":"10.1016\/j.preteyeres.2024.101299_bib89","doi-asserted-by":"crossref","first-page":"857","DOI":"10.1016\/j.jcf.2021.06.005","article-title":"Entry of cystic fibrosis transmembrane conductance potentiator ivacaftor into the developing brain and lung","volume":"20","author":"Qiu","year":"2021","journal-title":"J. Cyst. Fibros."},{"issue":"9","key":"10.1016\/j.preteyeres.2024.101299_bib90","doi-asserted-by":"crossref","DOI":"10.26508\/lsa.202101326","article-title":"Exploring YAP1-centered networks linking dysfunctional CFTR to epithelial-mesenchymal transition","volume":"5","author":"Quaresma","year":"2022","journal-title":"Life Sci. Alliance"},{"issue":"10","key":"10.1016\/j.preteyeres.2024.101299_bib91","doi-asserted-by":"crossref","first-page":"920","DOI":"10.1038\/s41419-020-03119-z","article-title":"Mutant CFTR Drives TWIST1 mediated epithelial-mesenchymal transition","volume":"11","author":"Quaresma","year":"2020","journal-title":"Cell Death Dis."},{"issue":"1","key":"10.1016\/j.preteyeres.2024.101299_bib92","doi-asserted-by":"crossref","first-page":"84","DOI":"10.1186\/s12967-017-1193-9","article-title":"Cystic fibrosis: current therapeutic targets and future approaches","volume":"15","author":"Rafeeq","year":"2017","journal-title":"J. Transl. Med."},{"issue":"5","key":"10.1016\/j.preteyeres.2024.101299_bib93","doi-asserted-by":"crossref","DOI":"10.1183\/13993003.02443-2019","article-title":"Re-imagining cystic fibrosis care: next generation thinking","volume":"55","author":"Rang","year":"2020","journal-title":"Eur. Respir. J."},{"issue":"1","key":"10.1016\/j.preteyeres.2024.101299_bib94","doi-asserted-by":"crossref","first-page":"35","DOI":"10.1038\/ng0593-35","article-title":"Production of a severe cystic fibrosis mutation in mice by gene targeting","volume":"4","author":"Ratcliff","year":"1993","journal-title":"Nat. Genet."},{"issue":"1","key":"10.1016\/j.preteyeres.2024.101299_bib95","doi-asserted-by":"crossref","first-page":"C132","DOI":"10.1152\/ajpcell.00201.2004","article-title":"Release of ATP from retinal pigment epithelial cells involves both CFTR and vesicular transport","volume":"288","author":"Reigada","year":"2005","journal-title":"Am. J. Physiol. Cell Physiol."},{"issue":"Suppl. 1","key":"10.1016\/j.preteyeres.2024.101299_bib96","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1186\/s12886-015-0135-7","article-title":"Ocular transient receptor potential channel function in health and disease","volume":"15","author":"Reinach","year":"2015","journal-title":"BMC Ophthalmol."},{"issue":"2","key":"10.1016\/j.preteyeres.2024.101299_bib97","doi-asserted-by":"crossref","first-page":"282","DOI":"10.1016\/j.jcf.2014.07.014","article-title":"Retinal screening of patients with cystic fibrosis-related diabetes in Wales -- a real eye opener","volume":"14","author":"Roberts","year":"2015","journal-title":"J. Cyst. Fibros."},{"issue":"4","key":"10.1016\/j.preteyeres.2024.101299_bib98","doi-asserted-by":"crossref","first-page":"202","DOI":"10.1159\/000309944","article-title":"Tear mucus crystallization in children with cystic fibrosis","volume":"197","author":"Rolando","year":"1988","journal-title":"Ophthalmologica"},{"issue":"Pt 20","key":"10.1016\/j.preteyeres.2024.101299_bib99","first-page":"4396","article-title":"CFTR interacts with ZO-1 to regulate tight junction assembly and epithelial differentiation through the ZONAB pathway","volume":"127","author":"Ruan","year":"2014","journal-title":"J. Cell Sci."},{"issue":"5","key":"10.1016\/j.preteyeres.2024.101299_bib100","doi-asserted-by":"crossref","DOI":"10.1016\/j.xops.2024.100475","article-title":"Label-free imaging of inflammation at the level of single cells in the living human eye","volume":"4","author":"Rui","year":"2024","journal-title":"Ophthalmol Sci"},{"issue":"1","key":"10.1016\/j.preteyeres.2024.101299_bib101","doi-asserted-by":"crossref","first-page":"93","DOI":"10.1007\/s00018-016-2391-y","article-title":"Role of CFTR in epithelial physiology","volume":"74","author":"Saint-Criq","year":"2017","journal-title":"Cell. Mol. Life Sci."},{"issue":"3","key":"10.1016\/j.preteyeres.2024.101299_bib102","doi-asserted-by":"crossref","first-page":"184","DOI":"10.3109\/13816810.2013.811270","article-title":"Familial exudative vitreoretinopathy caused by a homozygous mutation in TSPAN12 in a cystic fibrosis infant","volume":"35","author":"Savarese","year":"2014","journal-title":"Ophthalmic Genet."},{"issue":"1","key":"10.1016\/j.preteyeres.2024.101299_bib103","doi-asserted-by":"crossref","first-page":"130","DOI":"10.1002\/cpt.548","article-title":"Can cystic fibrosis patients finally catch a breath with lumacaftor\/ivacaftor?","volume":"101","author":"Schneider","year":"2017","journal-title":"Clin. Pharmacol. Ther."},{"issue":"9","key":"10.1016\/j.preteyeres.2024.101299_bib104","doi-asserted-by":"crossref","first-page":"3256","DOI":"10.1073\/pnas.0800254105","article-title":"Phenylalanine-508 mediates a cytoplasmic-membrane domain contact in the CFTR 3D structure crucial to assembly and channel function","volume":"105","author":"Serohijos","year":"2008","journal-title":"Proc. Natl. Acad. Sci. U.S.A."},{"issue":"9","key":"10.1016\/j.preteyeres.2024.101299_bib105","doi-asserted-by":"crossref","first-page":"1160","DOI":"10.1038\/eye.2016.156","article-title":"Global prevalence of childhood cataract: a systematic review","volume":"30","author":"Sheeladevi","year":"2016","journal-title":"Eye"},{"issue":"11","key":"10.1016\/j.preteyeres.2024.101299_bib106","doi-asserted-by":"crossref","first-page":"1624","DOI":"10.1016\/S0161-6420(89)32676-5","article-title":"The ocular surface in cystic fibrosis","volume":"96","author":"Sheppard","year":"1989","journal-title":"Ophthalmology"},{"issue":"1","key":"10.1016\/j.preteyeres.2024.101299_bib107","doi-asserted-by":"crossref","first-page":"1","DOI":"10.18332\/pne\/157586","article-title":"Pregnancy in cystic fibrosis in the era of CFTR modulators: a case series [journal article]","volume":"36","author":"Sionidou","year":"2023","journal-title":"Pneumon"},{"issue":"6","key":"10.1016\/j.preteyeres.2024.101299_bib108","doi-asserted-by":"crossref","first-page":"592","DOI":"10.1016\/S0161-6420(83)34512-7","article-title":"Effects of vitamin A deficiency on the ocular surface","volume":"90","author":"Sommer","year":"1983","journal-title":"Ophthalmology"},{"issue":"6","key":"10.1016\/j.preteyeres.2024.101299_bib109","doi-asserted-by":"crossref","first-page":"2803","DOI":"10.1167\/iovs.03-0001","article-title":"Aquaporin-1 channels in human retinal pigment epithelium: role in transepithelial water movement","volume":"44","author":"Stamer","year":"2003","journal-title":"Invest. Ophthalmol. Vis. Sci."},{"issue":"4","key":"10.1016\/j.preteyeres.2024.101299_bib110","doi-asserted-by":"crossref","first-page":"642","DOI":"10.1542\/peds.84.4.642","article-title":"Diabetic microangiopathy in patients with cystic fibrosis","volume":"84","author":"Sullivan","year":"1989","journal-title":"Pediatrics"},{"issue":"10","key":"10.1016\/j.preteyeres.2024.101299_bib111","doi-asserted-by":"crossref","first-page":"3325","DOI":"10.1210\/en.2017-00187","article-title":"CFTR influences beta cell function and insulin secretion through non-cell autonomous exocrine-derived factors","volume":"158","author":"Sun","year":"2017","journal-title":"Endocrinology"},{"issue":"4","key":"10.1016\/j.preteyeres.2024.101299_bib112","doi-asserted-by":"crossref","first-page":"C673","DOI":"10.1152\/ajpcell.00384.2001","article-title":"Expression, localization, and functional evaluation of CFTR in bovine corneal endothelial cells","volume":"282","author":"Sun","year":"2002","journal-title":"Am. J. Physiol. Cell Physiol."},{"issue":"1","key":"10.1016\/j.preteyeres.2024.101299_bib113","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1076\/ceyr.22.1.1.6981","article-title":"Studies on the expression of mRNA for anion transport related proteins in corneal endothelial cells","volume":"22","author":"Sun","year":"2001","journal-title":"Curr. Eye Res."},{"issue":"3","key":"10.1016\/j.preteyeres.2024.101299_bib114","doi-asserted-by":"crossref","first-page":"402","DOI":"10.1016\/j.jcf.2021.03.006","article-title":"Maternal and fetal outcomes following elexacaftor-tezacaftor-ivacaftor use during pregnancy and lactation","volume":"20","author":"Taylor-Cousar","year":"2021","journal-title":"J. Cyst. Fibros."},{"issue":"2","key":"10.1016\/j.preteyeres.2024.101299_bib115","doi-asserted-by":"crossref","DOI":"10.1183\/23120541.00082-2019","article-title":"Clinical development of triple-combination CFTR modulators for cystic fibrosis patients with one or two F508del alleles","volume":"5","author":"Taylor-Cousar","year":"2019","journal-title":"ERJ Open Res"},{"issue":"3","key":"10.1016\/j.preteyeres.2024.101299_bib116","doi-asserted-by":"crossref","first-page":"182","DOI":"10.1076\/ceyr.24.3.182.8297","article-title":"Presence of CFTR in the conjunctival epithelium","volume":"24","author":"Turner","year":"2002","journal-title":"Curr. Eye Res."},{"issue":"4","key":"10.1016\/j.preteyeres.2024.101299_bib117","doi-asserted-by":"crossref","first-page":"420","DOI":"10.1016\/j.preteyeres.2008.04.001","article-title":"Functions of aquaporins in the eye","volume":"27","author":"Verkman","year":"2008","journal-title":"Prog. Retin. Eye Res."},{"issue":"4","key":"10.1016\/j.preteyeres.2024.101299_bib118","doi-asserted-by":"crossref","first-page":"416","DOI":"10.1080\/02713683.2022.2156548","article-title":"Cystic fibrosis transmembrane conductance regulator attenuates oxidative stress-induced injury in diabetic retinopathy rats","volume":"48","author":"Wang","year":"2023","journal-title":"Curr. Eye Res."},{"issue":"8","key":"10.1016\/j.preteyeres.2024.101299_bib119","doi-asserted-by":"crossref","first-page":"3888","DOI":"10.1073\/pnas.0913001107","article-title":"ATP-independent CFTR channel gating and allosteric modulation by phosphorylation","volume":"107","author":"Wang","year":"2010","journal-title":"Proc. Natl. Acad. Sci. USA"},{"issue":"3","key":"10.1016\/j.preteyeres.2024.101299_bib120","doi-asserted-by":"crossref","first-page":"C839","DOI":"10.1152\/ajpcell.00445.2001","article-title":"Oxidant and antioxidant modulation of chloride channels expressed in human retinal pigment epithelium","volume":"283","author":"Weng","year":"2002","journal-title":"Am. J. Physiol. Cell Physiol."},{"key":"10.1016\/j.preteyeres.2024.101299_bib126","first-page":"1","article-title":"Redefining our vision: an updated guide to the ocular immune system","author":"Wu","year":"2024","journal-title":"Nature Reviews Immunology"},{"key":"10.1016\/j.preteyeres.2024.101299_bib121","doi-asserted-by":"crossref","DOI":"10.1016\/j.ophtha.2024.04.020","article-title":"Intravital imaging of the human cornea reveals the differential effects of season on innate and adaptive immune cell morphodynamics","author":"Wu","year":"2024","journal-title":"Ophthalmology"},{"key":"10.1016\/j.preteyeres.2024.101299_bib122","article-title":"Ocular surface ion-channels are closely related to dry eye: key research focus on innovative drugs for dry eye","volume":"9","author":"Yang","year":"2022","journal-title":"Front. Med."},{"key":"10.1016\/j.preteyeres.2024.101299_bib123","doi-asserted-by":"crossref","DOI":"10.3389\/fphar.2023.1265138","article-title":"Ocular development after highly effective modulator treatment early in life","volume":"14","author":"Zhu","year":"2023","journal-title":"Front. Pharmacol."}],"container-title":["Progress in Retinal and Eye Research"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1350946224000648?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1350946224000648?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2024,11,7]],"date-time":"2024-11-07T20:01:23Z","timestamp":1731009683000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S1350946224000648"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,11]]},"references-count":122,"alternative-id":["S1350946224000648"],"URL":"https:\/\/doi.org\/10.1016\/j.preteyeres.2024.101299","relation":{},"ISSN":["1350-9462"],"issn-type":[{"value":"1350-9462","type":"print"}],"subject":[],"published":{"date-parts":[[2024,11]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"The role of CFTR in the eye, and the effect of early highly effective modulator treatment for cystic fibrosis on eye health","name":"articletitle","label":"Article Title"},{"value":"Progress in Retinal and Eye Research","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.preteyeres.2024.101299","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2024 The Authors. Published by Elsevier Ltd.","name":"copyright","label":"Copyright"}],"article-number":"101299"}}