{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,24]],"date-time":"2026-08-24T12:24:56Z","timestamp":1787574296051,"version":"build-2736575974"},"reference-count":45,"publisher":"MDPI AG","issue":"17","license":[{"start":{"date-parts":[[2026,8,22]],"date-time":"2026-08-22T00:00:00Z","timestamp":1787356800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000738","name":"Department of Veterans Administration","doi-asserted-by":"publisher","award":["5I01BX001000-13"],"award-info":[{"award-number":["5I01BX001000-13"]}],"id":[{"id":"10.13039\/100000738","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000005","name":"Department of Defense Grant","doi-asserted-by":"publisher","award":["TS240031"],"award-info":[{"award-number":["TS240031"]}],"id":[{"id":"10.13039\/100000005","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100015502","name":"Dialysis Clinic, Inc. Grant","doi-asserted-by":"publisher","award":["C-4149"],"award-info":[{"award-number":["C-4149"]}],"id":[{"id":"10.13039\/100015502","id-type":"DOI","asserted-by":"publisher"}]},{"name":"NIH Grants","award":["R01ES34400"],"award-info":[{"award-number":["R01ES34400"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Int. J. Mol. Sci.","IJMS"],"abstract":"<jats:p>Kidney cystic epithelium primarily comprises proliferating A-intercalated (A-IC) cells in humans and mouse models of TSC. These studies explored the expression of transcription factors (TF) that drive the development of A-IC cells and the downregulation of B-intercalated (B-IC) cells, as well as the status and localization of Tsc1 and Tsc2 in epithelial cells lining the cysts. Transcriptome studies indicated enhanced expression of the following TFs: DMRT2, FOXI1, and FOXP1 in young TSC mice, and DMRT2 and FOXI1 in aged mice. The expression of Hmx2 decreased in TSC mice of all ages. Our studies further demonstrated: (1) distinct and predominant DMRT2 and FOXP1 localization in the cystic epithelium of in TSC mouse models; and (2) expression of Foxi1, Tsc1, and Tsc2 in epithelial cells lining the kidney cysts. Expression of DMRT2, FOXI1, and FOXP1 is enhanced in the A-IC cells lining the kidney cysts of TSC mouse models. In the same models, the expression of Hmx2 mRNA is decreased and is accompanied by the loss of B-IC cells in cyst epithelia. Both DMRT2 and FOXP1 localized to the nucleus of A-IC cells of the renal cysts, suggesting that TSC kidney cystogenesis is driven by factors that exclusively promote A-IC expansion.<\/jats:p>","DOI":"10.3390\/ijms27177515","type":"journal-article","created":{"date-parts":[[2026,8,24]],"date-time":"2026-08-24T11:47:19Z","timestamp":1787572039000},"page":"7515","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Kidney Cyst Epithelia in Tuberous Sclerosis Complex (TSC) Exhibit Propagation and Expansion of A-Intercalated, but Loss of B-Intercalated and Principal Cells: The Role of FOXI1, FOXP1, and DMRT2 Transcription Factors"],"prefix":"10.3390","volume":"27","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4909-4469","authenticated-orcid":false,"given":"Manoocher","family":"Soleimani","sequence":"first","affiliation":[{"name":"Research Services, New Mexico Veterans Health Care System, Albuquerque, NM 87108, USA"},{"name":"Department of Internal Medicine, University of New Mexico School of Medicine, Albuquerque, NM 87106, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4257-5107","authenticated-orcid":false,"given":"Kamyar","family":"Zahedi","sequence":"additional","affiliation":[{"name":"Research Services, New Mexico Veterans Health Care System, Albuquerque, NM 87108, USA"},{"name":"Department of Internal Medicine, University of New Mexico School of Medicine, Albuquerque, NM 87106, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6582-2281","authenticated-orcid":false,"given":"Marybeth","family":"Brooks","sequence":"additional","affiliation":[{"name":"Research Services, New Mexico Veterans Health Care System, Albuquerque, NM 87108, USA"},{"name":"Department of Internal Medicine, University of New Mexico School of Medicine, Albuquerque, NM 87106, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julie G.","family":"In","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, University of New Mexico School of Medicine, Albuquerque, NM 87106, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5710-292X","authenticated-orcid":false,"given":"Sharon","family":"Barone","sequence":"additional","affiliation":[{"name":"Research Services, New Mexico Veterans Health Care System, Albuquerque, NM 87108, USA"},{"name":"Department of Internal Medicine, University of New Mexico School of Medicine, Albuquerque, NM 87106, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2026,8,22]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1345","DOI":"10.1056\/NEJMra055323","article-title":"The tuberous sclerosis complex","volume":"355","author":"Crino","year":"2006","journal-title":"N. 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