@article{46cf784532394ef5bf9294c5a9159526,
title = "Tuberous sclerosis complex exhibits a new renal cystogenic mechanism",
abstract = "Tuberous sclerosis complex (TSC) is a tumor predisposition syndrome with significant renal cystic and solid tumor disease. While the most common renal tumor in TSC, the angiomyolipoma, exhibits a loss of heterozygosity associated with disease, we have discovered that the renal cystic epithelium is composed of type A intercalated cells that have an intact Tsc gene that have been induced to exhibit Tsc-mutant disease phenotype. This mechanism appears to be different than that for ADPKD. The murine models described here closely resemble the human disease and both appear to be mTORC1 inhibitor responsive. The induction signaling driving cystogenesis may be mediated by extracellular vesicle trafficking.",
keywords = "Intercalated cells, Tuberous sclerosis complex, renal cystic disease, renal cystogenesis",
author = "Bissler, {John J.} and Fahad Zadjali and Dave Bridges and Aristotelis Astrinidis and Sharon Barone and Ying Yao and Redd, {Je Anna R.} and Siroky, {Brian J.} and Yanqing Wang and Finley, {Joel T.} and Rusiniak, {Michael E.} and Heinz Baumann and Kamyar Zahedi and Gross, {Kenneth W.} and Manoocher Soleimani",
note = "Funding Information: Funding Information This work was supported by DoD grant W81XWH-14-1-0343(JJB), NIH grant DK107535, Le Bonheur Grant 650700 and funds from the Memphis Research Consortium (DB), NIH Grant HL48459(KWG) and NIH R21 12121(KWG), Veterans Administration (5 I01 BX001000-06) and Center for Genetics of Transport and Epithelial Biology (MS). This work utilized core facilities supported by Roswell Park Cancer Institute and NIH grant P30CA016056. This work is also supported by the Agliata family in memory of Jacob. Publisher Copyright: {\textcopyright} 2019 The Authors. Physiological Reports published by Wiley Periodicals, Inc. on behalf of The Physiological Society and the American Physiological Society.",
year = "2019",
month = jan,
doi = "10.14814/phy2.13983",
language = "English",
volume = "7",
journal = "Physiological Reports",
issn = "2051-817X",
publisher = "John Wiley and Sons Inc.",
number = "2",
}