Wilms tumor protein
| Wilms tumor protein | ||
|---|---|---|
| Properties of human protein | ||
| Mass / length primary structure | 449 amino acids | |
| Cofactor | Zn 2+ | |
| Isoforms | 5 | |
| Identifier | ||
| Gene name | WT1 | |
| External IDs | ||
| Occurrence | ||
| Homology family | Wilms tumor protein | |
| Parent taxon | Euteleostomi | |
The Wilms tumor protein (WT) is a protein in vertebrates that, as a transcription factor, controls the activity of other genes. The WT protein can have both an activating and a repressive effect. WTP is mainly expressed in the kidneys and in some of the blood-forming cells.
The associated gene, the Wilms tumor suppressor gene ( WT1 ), is found on band 13 of the short arm of chromosome 11 in humans .
Mutations in WT1 - gene cause Wilms tumors (named after the surgeon Max Wilms ), a kidney tumor that occurs with a frequency of about 1-9 in 100,000 in children up to 15 years. Furthermore, WT1 mutations can lead to Frasier syndrome , Denys-Drash syndrome , Meacham syndrome or hypospadias .