Gene Expression Profiling of Glomeruli from a mouse model of Denys-Drash Syndrome

The Wilms tumor-suppressor gene WT1, a key player in renal development, also has a crucial role in maintenance of the glomerulus in the mature kidney. However, molecular pathways orchestrated by WT1 in podocytes, where it is highly expressed, remain unknown. Their defects are thought to modify the cross-talk between podocytes and other glomerular cells and ultimately lead to glomerular sclerosis, as observed in diffuse mesangial sclerosis (DMS) a nephropathy associated with WT1 mutations.

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Ratelade J, Arrondel C, Hamard G, Garbay S, Harvey S, Biebuyck N, Schulz H, Hastie N, Pontoglio M, Gubler MC, Antignac C, Heidet L