Submitted on December 17, 2007
Accepted on March 28, 2008
New insights into the function of the Wilms tumour suppressor gene WT1 in podocytes
Avril A Morrison1, Rebecca L Viney2, Moin A. Saleem3, and Michael Robert Ladomery4*
1 Department of Clinical Sciences at North Bristol, University of Bristol, Bristol, England, United Kingdom
2 Faculty of Health and Life Sciences, University of the West of England, Bristol, England, United Kingdom
3 Academic Renal Unit, University of Bristol, Bristol, England, United Kingdom
4 Faculty of Health and Life Sciences, University of the West of England, Bristol, England, United Kingdom; , United Kingdom
* To whom correspondence should be addressed. E-mail: Michael.Ladomery{at}uwe.ac.uk.
The Wilms tumour 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 (Wilms tumour, Aniridia, Genitourinary anomalies and mental Retardation), Frasier and Denys-Drash Syndromes. Recent advances including the development of transgenic mouse models and conditionally immortalised podocyte cell lines are beginning to shed light on WT1 s crucial role in podocyte function.
