{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,10]],"date-time":"2026-07-10T13:17:34Z","timestamp":1783689454109,"version":"3.55.0"},"reference-count":67,"publisher":"Public Library of Science (PLoS)","issue":"1","license":[{"start":{"date-parts":[[2024,1,22]],"date-time":"2024-01-22T00:00:00Z","timestamp":1705881600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100001209","name":"Knights Templar Eye Foundation","doi-asserted-by":"publisher","award":["Career Starter Grant"],"award-info":[{"award-number":["Career Starter Grant"]}],"id":[{"id":"10.13039\/100001209","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Walton Li Chair in Cornea and Uveitis"},{"DOI":"10.13039\/100000053","name":"National Eye Institute","doi-asserted-by":"publisher","award":["P30EY000331"],"award-info":[{"award-number":["P30EY000331"]}],"id":[{"id":"10.13039\/100000053","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Stotter Revocable Trust"},{"DOI":"10.13039\/100001818","name":"Research to Prevent Blindness","doi-asserted-by":"publisher","award":["Unrestricted grant to Stein Eye Institute"],"award-info":[{"award-number":["Unrestricted grant to Stein Eye Institute"]}],"id":[{"id":"10.13039\/100001818","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["www.plosone.org"],"crossmark-restriction":false},"short-container-title":["PLoS ONE"],"abstract":"<jats:p>Mutations in the solute linked carrier family 4 member 11 (<jats:italic>SLC4A11)<\/jats:italic> gene are associated with congenital hereditary endothelial dystrophy (CHED) and Fuchs corneal endothelial dystrophy type 4 (FECD4), both characterized by corneal endothelial cell (CEnC) dysfunction and\/or cell loss leading to corneal edema and visual impairment. In this study, we characterize the impact of CHED-\/FECD4-associated <jats:italic>SLC4A11<\/jats:italic> mutations on CEnC function and SLC4A11 protein localization by generating and comparing human CEnC (hCEnC) lines expressing wild type SLC4A11 (SLC4A11<jats:sup>WT<\/jats:sup>) or mutant <jats:italic>SLC4A11<\/jats:italic> harboring CHED-\/FECD4-associated SLC4A11 mutations (SLC4A11<jats:sup>MU<\/jats:sup>). SLC4A11<jats:sup>WT<\/jats:sup> and SLC4A11<jats:sup>MU<\/jats:sup> hCEnC lines were generated to express either SLC4A11 variant 2 (V2<jats:sup>WT<\/jats:sup> and V2<jats:sup>MU<\/jats:sup>) or variant 3 (V3<jats:sup>WT<\/jats:sup> and V3<jats:sup>MU<\/jats:sup>), the two major variants expressed in ex vivo hCEnC. Functional assays were performed to assess cell barrier, proliferation, viability, migration, and NH<jats:sub>3<\/jats:sub>-induced membrane conductance. We demonstrate SLC4A11<jats:sup>-\/-<\/jats:sup> and SLC4A11<jats:sup>MU<\/jats:sup> hCEnC lines exhibited increased migration rates, altered proliferation and decreased cell viability compared to <jats:italic>SLC4A11<\/jats:italic><jats:sup>WT<\/jats:sup> hCEnC. Additionally, SLC4A11<jats:sup>-\/-<\/jats:sup> hCEnC demonstrated decreased cell-substrate adhesion and membrane capacitances compared to SLC4A11<jats:sup>WT<\/jats:sup> hCEnC. Induction with 10mM NH<jats:sub>4<\/jats:sub>Cl led SLC4A11<jats:sup>WT<\/jats:sup> hCEnC to depolarize; conversely, SLC4A11<jats:sup>-\/-<\/jats:sup> hCEnC hyperpolarized and the majority of SLC4A11<jats:sup>MU<\/jats:sup> hCEnC either hyperpolarized or had minimal membrane potential changes following NH<jats:sub>4<\/jats:sub>Cl induction. Immunostaining of primary hCEnC and SLC4A11<jats:sup>WT<\/jats:sup> hCEnC lines for SLC4A11 demonstrated predominately plasma membrane staining with poor or partial colocalization with mitochondrial marker COX4 within a subset of punctate subcellular structures. Overall, our findings suggest CHED-associated <jats:italic>SLC4A11<\/jats:italic> mutations likely lead to hCEnC dysfunction, and ultimately CHED, by interfering with cell migration, proliferation, viability, membrane conductance, barrier function, and\/or cell surface localization of the SLC4A11 protein in hCEnC. Additionally, based on their similar subcellular localization and exhibiting similar cell functional profiles, protein isoforms encoded by <jats:italic>SLC4A11<\/jats:italic> variant 2 and variant 3 likely have highly overlapping functional roles in hCEnC.<\/jats:p>","DOI":"10.1371\/journal.pone.0296928","type":"journal-article","created":{"date-parts":[[2024,1,22]],"date-time":"2024-01-22T18:26:41Z","timestamp":1705948001000},"page":"e0296928","update-policy":"https:\/\/doi.org\/10.1371\/journal.pone.corrections_policy","source":"Crossref","is-referenced-by-count":4,"title":["Investigation of the functional impact of CHED- and FECD4-associated SLC4A11 mutations in human corneal endothelial cells"],"prefix":"10.1371","volume":"19","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8505-9495","authenticated-orcid":true,"given":"Doug D.","family":"Chung","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Angela C.","family":"Chen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Charlene H.","family":"Choo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3229-9177","authenticated-orcid":true,"given":"Wenlin","family":"Zhang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dominic","family":"Williams","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christopher G.","family":"Griffis","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul","family":"Bonezzi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kavya","family":"Jatavallabhula","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alapakkam P.","family":"Sampath","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9082-4796","authenticated-orcid":true,"given":"Anthony J.","family":"Aldave","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"340","published-online":{"date-parts":[[2024,1,22]]},"reference":[{"issue":"2","key":"pone.0296928.ref001","doi-asserted-by":"crossref","first-page":"117","DOI":"10.1097\/ICO.0000000000000307","article-title":"IC3D classification of corneal dystrophies\u2014edition 2","volume":"34","author":"JS Weiss","year":"2015","journal-title":"Cornea"},{"issue":"7","key":"pone.0296928.ref002","doi-asserted-by":"crossref","first-page":"755","DOI":"10.1038\/ng1824","article-title":"Mutations in sodium-borate cotransporter SLC4A11 cause recessive congenital hereditary endothelial dystrophy (CHED2).","volume":"38","author":"EN Vithana","year":"2006","journal-title":"Nat Genet"},{"issue":"7","key":"pone.0296928.ref003","doi-asserted-by":"crossref","first-page":"896","DOI":"10.1097\/ICO.0b013e318074bb01","article-title":"Autosomal recessive CHED associated with novel compound heterozygous mutations in SLC4A11","volume":"26","author":"AJ Aldave","year":"2007","journal-title":"Cornea"},{"issue":"5","key":"pone.0296928.ref004","doi-asserted-by":"crossref","first-page":"322","DOI":"10.1136\/jmg.2006.046904","article-title":"Borate transporter SLC4A11 mutations cause both Harboyan syndrome and non-syndromic corneal endothelial dystrophy","volume":"44","author":"J Desir","year":"2007","journal-title":"J Med Genet"},{"issue":"1","key":"pone.0296928.ref005","doi-asserted-by":"crossref","first-page":"64","DOI":"10.1136\/jmg.2006.044644","article-title":"Autosomal recessive corneal endothelial dystrophy (CHED2) is associated with mutations in SLC4A11.","volume":"44","author":"X Jiao","year":"2007","journal-title":"J Med Genet"},{"key":"pone.0296928.ref006","first-page":"39","article-title":"Genetic analysis of two Indian families affected with congenital hereditary endothelial dystrophy: two novel mutations in SLC4A11","volume":"13","author":"A Kumar","year":"2007","journal-title":"Mol Vis"},{"issue":"5","key":"pone.0296928.ref007","doi-asserted-by":"crossref","first-page":"522","DOI":"10.1002\/humu.9487","article-title":"Novel SLC4A11 mutations in patients with recessive congenital hereditary endothelial dystrophy (CHED2). 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