Pulmonary dysplasia, Denys-Drash syndrome and Wilms tumor 1 gene mutation in twins
β Scribed by Vikas R. Dharnidharka; E. Cristy Ruteshouser; Seymour Rosen; Harry Kozakewich; H. William Harris Jr.; John T. Herrin; Vicki Huff
- Publisher
- Springer
- Year
- 2001
- Tongue
- English
- Weight
- 100 KB
- Volume
- 16
- Category
- Article
- ISSN
- 0931-041X
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Denys-Drash syndrome (DDS) is characterized by early onset nephropathy, pseudohermaphroditism in males and a high risk for developing Wilm's tumour (WT). The exact cause of DDS is unknown but germline mutations in the Wilm's tumour suppressor gene (WT1) have recently been described in the majority o
## Abstract The Wilms' tumor 1 gene, __WT1__, encodes a zincβfinger protein that is implicated in the development of Wilms' tumor. Mutant or aberrantly expressed WT1 isoforms have also been described in desmoplastic small round cell tumor, acute leukemias, mesothelioma, breast tumors and melanoma.