WT-1 (-KTS) human

Wilms tumor 1 (WT1) is encoded by the gene mapped to human chromosome 11p13. The encoded protein is expressed at high levels in nephrons, but at low levels in gonads, visceral adipose tissue, mesothelium and bone marrow cells. In addition, it is also abundantly expressed in renal podocytes in adults and in parietal epithelial cells during early days.

WT-1, the product of Wilms′ tumor suppressor gene Wt1, is a nuclear protein with structural motifs characteristic of transcription factors, including four C-terminal zinc fingers. While different pre-mRNA processing could result in 16 isoforms of the protein, inclusion or exclusion of exon 5 and the three amino acids (KTS) between zinc fingers 3 and 4 largely affects the activity of WT1 protein. Such a complex post-transcriptional regulation, particularly in splicing, may represent a major regulatory mechanism for tumorigenesis of the Wilms′ tumor. WT1 (-KTS) appears to have different binding affinity to both DNA and RNA compared to the +KTS form.

Wilms tumor 1 (WT1) is involved in various vertebrate developmental processes such as cell differentiation and proliferation, apoptosis and regulation of epithelial/mesenchymal characteristics. It serves as a tumor suppressor gene but might also possess oncogenic property. The encoded protein regulates mRNA splicing and interactions between protein. It is associated with cancers and as well as kidney function. Mutations in the gene leads to Denys-Drash syndrome, resulting in renal and genitourinary tract abnormalities. Deletion in the germline WT1 gene causes Wilms′ tumor. This gene is highly expressed in a various types of cancers.

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