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Complete sex reversal in a WAGR syndrome patient

✍ Scribed by Cedric Le Caignec; Capucine Delnatte; Joris R. Vermeesch; Michelle Boceno; Madeleine Joubert; Francoise Lavenant; Albert David; Jean-Marie Rival

Publisher
John Wiley and Sons
Year
2007
Tongue
English
Weight
107 KB
Volume
143A
Category
Article
ISSN
1552-4825

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## Abstract Wilms tumor, aniridia, genitourinary anomalies, and mental retardation (WAGR) syndrome is a contiguous gene deletion syndrome involving the Wilms tumor 1 gene (__WT1__), the paired box gene 6 (__PAX6__), and possibly other genes on chromosome 11p13. __WT1__ is required for normal format

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Wilms' tumor is a childhood nephroblastoma that is postulated to arise through the inactivation of a tumor suppressor gene by a two-hit mechanism. A candidate I I p I 3 Wilms' tumor gene, WTI. has been cloned and shown to encode a zinc finger protein. Patients with the WAGR syndrome (Wilms' tumor, a