Wilms tumor protein Simoa

Wilms tumor protein (WT-1) is a transcription factor encoded by the WT1 gene on chromosome 11p. The protein contains four zinc-finger motifs at the C-terminus and a proline/glutamine rich DNA-binding domain at the N-terminus which plays an important role in cellular development and cell survival. It recognizes and binds to the DNA sequence 5-GCG(T/G)GGGCG-3, regulates the expression of numerous target genes, including EPO, and plays an essential role for development of the urogenital system. WT-1 has a tumor suppressor as well as an oncogenic role in tumor formation. The protein also seems to be involved in tumorigenesis, as WT-1 is overexpressed in most cases of AML, CML, MDS and acute lymphoblastic leukemia (ALL), and in several solid tumors. Further, expression increases as disease progresses, making WT-1 an attractive vaccine candidate in otherwise untreatable advanced leukemias. WT-1 may be isoform-specific: isoforms lacking the KTS motif may act as transcription factors; isoforms containing the KTS motif may bind mRNA and play a role in mRNA metabolism or splicing.

Swiss-Prot Accession Number: P19544