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INVITED REVIEW

New insights into the function of the Wilms tumor suppressor gene WT1 in podocytes

¹Bristol Genomics Research Institute, Centre for Research in Biomedicine, Faculty of Health and Life Sciences, University of the West of England, and ²Academic and Children's Renal Unit, Lifeline Building, Southmead Hospital, University of Bristol, Bristol, United Kingdom

Submitted 17 December 2007 ; accepted in final form 28 March 2008

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.