Denys-Drash syndrome is a condition that affects the kidneys and genitalia.
Mutations in the WT1 gene cause Denys-Drash syndrome. The WT1 gene provides instructions for making a protein that regulates the activity of other genes by attaching (binding) to specific regions of DNA. On the basis of this action, the WT1 protein is called a transcription factor. The WT1 protein plays a role in the development of the kidneys and gonads (ovaries in females and testes in males) before birth.
The prevalence of Denys-Drash syndrome is unknown; at least 150 affected individuals have been reported in the scientific literature.
This condition is inherited in an autosomal dominant pattern, which means one copy of the altered gene in each cell is sufficient to cause the disorder.
Published Date: March 01, 2013Published By: National Institutes of Health