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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

Avril A. Morrison

Rebecca L. Viney

Moin A. Saleem



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
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