F. Poulat, D. Morin, A. Konig, P. Brun, J. Giltay, C. Sultan, R. Dumas, Manfred Gessler, P. Berta

• The direct involvment of the Wilm's tumor suppressor gene (WTl) in Denys-Drash syndrome through mutations within exons 8 or 9 has recently been established. The absence of such alterations in three patients with Frasier syndrome provides a molecular basis for distinguishing these two syndromes that are associated with streak gonads, pseudohermaphroditism and renal failure.