Avril A. Morrison
New insights into the function of the Wilms tumor suppressor gene WT1 in podocytes
Morrison, Avril A.; Viney, Rebecca L.; Saleem, Moin A.; Ladomery, Michael
Authors
Rebecca L. Viney
Moin A. Saleem
Prof Michael Ladomery Michael.Ladomery@uwe.ac.uk
Professor of Genetics
Abstract
The Wilms tumor suppressor gene WT1 is essential for early urogenital development: homozygous mutations in WT1 result in embryonic lethality due to a failure in the development of kidneys and gonads. In the adult kidney, WT1 expression is limited to the glomerular podocytes. Several human nephrotic diseases arise from mutations of the WT1 gene, including mutations that affect its zinc-fingers and alternative splicing of +/-KTS isoforms. These include WAGR (for Wilms tumor, aniridia, genitourinary anomalies, and mental retardation), and Frasier and Denys-Drash syndromes. Recent advances including the development of transgenic mouse models and conditionally immortalized podocyte cell lines are beginning to shed light on WT1's crucial role in podocyte function. Copyright © 2008 the American Physiological Society.
Citation
Morrison, A. A., Viney, R. L., Saleem, M. A., & Ladomery, M. (2008). New insights into the function of the Wilms tumor suppressor gene WT1 in podocytes. AJP - Renal Physiology, 295(1), F12-F17. https://doi.org/10.1152/ajprenal.00597.2007
Journal Article Type | Review |
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Publication Date | Jul 1, 2008 |
Journal | American Journal of Physiology - Renal Physiology |
Print ISSN | 1931-857X |
Electronic ISSN | 1522-1466 |
Publisher | American Physiological Society |
Peer Reviewed | Peer Reviewed |
Volume | 295 |
Issue | 1 |
Pages | F12-F17 |
DOI | https://doi.org/10.1152/ajprenal.00597.2007 |
Keywords | Wilms tumor suppressor gene, WT1, podocytes |
Public URL | https://uwe-repository.worktribe.com/output/1013531 |
Publisher URL | http://dx.doi.org/10.1152/ajprenal.00597.2007 |
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