Last reviewed 01/2018
The Wilms' tumour gene (WT1) is a tumour suppressor which resides at chromosome 11p13. The protein encoded by the WT1 gene is a transcriptional repressor that downregulates expression of the insulin-like growth factor II and the insulin-like growth factor I receptor.
Differential splicing of the WT1 transcript results in four isoforms of the final protein.
All isoforms have four zinc finger DNA-binding domains but they have differing sequence specificities.
WT1 is expressed in:
- nephrogenesis - in the condensed mesenchyme, renal vesicle and glomerular epithelium
- the indifferent gonad, eventually being restricted to the Sertoli cells of the testis and granulosa and epithelial cells of the ovary, and the uterus
- WT1 is also one of the molecules that are known to control cellular apoptosis (3)
- Breuning, W. et al.. Nature Genetics1992; 1: 144-8
- Skuse GR, Ludlow JW. Tumour suppressor genes in disease and therapy. Lancet 1995;345: 902-6.
- Yang L, Han Y, Suarez Saiz F, Minden MD. A tumor suppressor and oncogene: the WT1 story. Leukemia 2007;21: 868-876.