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We report the generation and characterization of a novel dRTA mutant mouse line, Ae1 L919X knock-in (KI). Homozygous L919X KI mice exhibit typical dRTA features, including reduced ability to acidify urine in response to an acid load. This renal acidification defect was associated with a reduced number of kAE1-positive type A intercalated cells. To assess whether these mice exhibit urinary sodium wasting, homozygous L919X KI mice and the previously described R607H KI mice were fed a salt-depleted acid diet. In line with human patients, both mouse strains exhibited urinary sodium loss. Additionally, we identified increased expression of tight junction proteins claudin 4 and claudin 10b, suggesting a compensatory paracellular pathway. Consistent with data from human patients, L919X KI mice displayed a milder phenotype than that of R607H KI mice. Our findings reveal that both mouse strains are appropriate models for dRTA with a urinary salt-wasting phenotype and compensatory upregulation of the paracellular pathway.<\/jats:p>","DOI":"10.1242\/dmm.052138","type":"journal-article","created":{"date-parts":[[2025,4,28]],"date-time":"2025-04-28T06:31:36Z","timestamp":1745821896000},"source":"Crossref","is-referenced-by-count":6,"title":["Urinary sodium wasting and disrupted collecting duct function in mice with distal renal tubular acidosis mutations"],"prefix":"10.1242","volume":"18","author":[{"given":"Priyanka","family":"Mungara","sequence":"first","affiliation":[{"name":"University of Alberta 1 Department of Physiology , , Edmonton, AB T6G2H7 ,","place":["Canada"]},{"name":"Membrane Protein Disease Research Group, University of Alberta 2 , Edmonton, AB T6G2H7 ,","place":["Canada"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kristina","family":"MacNaughton","sequence":"additional","affiliation":[{"name":"University of Alberta 1 Department of Physiology , , Edmonton, AB T6G2H7 ,","place":["Canada"]},{"name":"Membrane Protein Disease Research Group, University of Alberta 2 , Edmonton, AB T6G2H7 ,","place":["Canada"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A. K. M. Shahid","family":"Ullah","sequence":"additional","affiliation":[{"name":"University of Alberta 1 Department of Physiology , , Edmonton, AB T6G2H7 ,","place":["Canada"]},{"name":"Membrane Protein Disease Research Group, University of Alberta 2 , Edmonton, AB T6G2H7 ,","place":["Canada"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Grace","family":"Essuman","sequence":"additional","affiliation":[{"name":"University of Alberta 1 Department of Physiology , , Edmonton, AB T6G2H7 ,","place":["Canada"]},{"name":"Membrane Protein Disease Research Group, University of Alberta 2 , Edmonton, AB T6G2H7 ,","place":["Canada"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Forough","family":"Chelangarimiyandoab","sequence":"additional","affiliation":[{"name":"University of Alberta 1 Department of Physiology , , Edmonton, AB T6G2H7 ,","place":["Canada"]},{"name":"Membrane Protein Disease Research Group, University of Alberta 2 , Edmonton, AB T6G2H7 ,","place":["Canada"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rizwan","family":"Mumtaz","sequence":"additional","affiliation":[{"name":"Institute of Human Genetics, Jena University Hospital, Friedrich Schiller University 3 , 07747 Jena ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J. Christopher","family":"Hennings","sequence":"additional","affiliation":[{"name":"Institute of Human Genetics, Jena University Hospital, Friedrich Schiller University 3 , 07747 Jena ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christian A.","family":"H\u00fcbner","sequence":"additional","affiliation":[{"name":"Institute of Human Genetics, Jena University Hospital, Friedrich Schiller University 3 , 07747 Jena ,","place":["Germany"]},{"name":"Jena University Hospital, Friedrich Schiller University 4 Center for Rare Diseases , , 07747 Jena ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dominique","family":"Eladari","sequence":"additional","affiliation":[{"name":"CHU Amiens Picardie, Facult\u00e9 de M\u00e9decine d'Amiens, Universit\u00e9 de Picardie Jules Verne 5 Service de M\u00e9decine de Pr\u00e9cision des Maladies R\u00e9nales et M\u00e9taboliques , , 80054 Amiens ,","place":["France"]},{"name":"Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale U970, Paris centre de recherche cardiovasculaire 6 (PARCC), 75015 Paris ,","place":["France"]},{"name":"French Clinical Research Infrastructure Network (F-CRIN) 7 , Investigation Network Initiative Cardiovascular and Renal Clinical Trialist ( ), 54500 Vand\u0153uvre-l\u00e8s-Nancy ,","place":["France"]},{"name":"INI-CRCT 7 , Investigation Network Initiative Cardiovascular and Renal Clinical Trialist ( ), 54500 Vand\u0153uvre-l\u00e8s-Nancy ,","place":["France"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R. Todd","family":"Alexander","sequence":"additional","affiliation":[{"name":"University of Alberta 1 Department of Physiology , , Edmonton, AB T6G2H7 ,","place":["Canada"]},{"name":"Membrane Protein Disease Research Group, University of Alberta 2 , Edmonton, AB T6G2H7 ,","place":["Canada"]},{"name":"University of Alberta 8 Department of Pediatric s, , Edmonton, AB T6G2H7 ,","place":["Canada"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emmanuelle","family":"Cordat","sequence":"additional","affiliation":[{"name":"University of Alberta 1 Department of Physiology , , Edmonton, AB T6G2H7 ,","place":["Canada"]},{"name":"Membrane Protein Disease Research Group, University of Alberta 2 , Edmonton, AB T6G2H7 ,","place":["Canada"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"237","published-online":{"date-parts":[[2025,5,23]]},"reference":[{"key":"2025052305372862000_DMM052138C1","doi-asserted-by":"publisher","first-page":"2243","DOI":"10.1111\/apa.15269","article-title":"Incomplete distal renal tubular acidosis in children","volume":"109","author":"Alonso-Varela","year":"2020","journal-title":"Acta Paediatr."},{"key":"2025052305372862000_DMM052138C2","first-page":"S57","article-title":"Familial renal tubular acidosis","volume":"23","author":"Alper","year":"2010","journal-title":"J. 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