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Denys-Drash Syndrome
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Definition
: A disorder of sex development characterized by UROGENITAL ABNORMALITIES; GONADAL DYSGENESIS; and WILMS TUMOR. It is caused by a mutation in the Wilms tumor suppressor gene (GENES, WILMS TUMOR) on chromosome 11.
See Also
Genes, Wilms Tumor
Other names
Syndrome, Drash; Syndrome, Denys-
Drash; Denys Drash Syndrome; Wilms Tumor and Pseudohermaphroditism; Pseudohermaphroditism, Nephron Disorder and Wilms'; Nephropathy, Wilms Tumor, and Genital Anomalies; Drash Syndrome
Substance
CAS Registry & name
Categories
Source
Fras1 protein, mouse
0
*Extracellular Matrix Proteins
Denys-Drash Syndrome.
Nat Genet 2004 Feb;36(2):172-7
