Anti-Wilms Tumor (WT33) from rabbit, purified by affinity chromatography

Wilms tumor protein (UniProt: P19544; also known as WT33) is encoded by the WT1 gene (Gene ID: 7490) in human. WT33 serves as a transcription factor that plays an important role in cellular development and cell survival. It is expressed in the kidney and a subset of hematopoietic cells. It regulates the expression of numerous target genes, including EPO. WT33 plays an essential role for development of the urogenital system and has a tumor suppressor as well as an oncogenic role in tumor formation. Eight different isoforms of WT33 have been described that are produced by alternative splicing. Isoforms lacking the KTS motif act as transcription factors while isoforms containing the KTS motif may bind mRNA and play a role in mRNA metabolism or splicing. Isoform 1 has lower affinity for DNA, and can bind RNA. The N-terminal region of WT33 contains a proline-rich region (aa 27-83) involved in transcriptional regulation, self-association, and RNA recognition, while its C-terminal region contains four C2H2-type zinc fingers (aa 323-347, 353-377, 383-405, 414-438) that mediate DNA and RNA binding. The zinc finger domain of WT33 can bind to GC-rich sequences, such as the EGR-1 consensus sequence (5 -GCG(T/G)GGGCG-3 ), the WTE motif (5'-GCGTGGGAGT-3'), or (TCC)n motif. Mutations in WT1 gene are reported to cause Wilms tumors characterized by embryonal malignancy of the kidney. Some mutations are reported to cause nephrotic syndrome 4, which causes severe proteinuria, resulting in complications such as hypoalbuminemia, hyperlipidemia and edema.