Wilms' tumour gene and function

The Wilms' tumour gene, WT1, encodes a protein with four zinc fingers that is probably a transcription factor. In humans, WT1 mutations can lead to childhood kidney tumours and to developmental defects of the kidney and gonad. The WT1 gene may have a role in the mesenchyme to epithelial switch in a range of mesodermally derived tissues. Furthermore, growth-factor genes may be targets for repression by the WT1 protein during development. WT1 is the first example of a tumour-suppressor gene with a specific developmental role.