Wilms Tumor Protein: Role, Structure and Implications Of Tumor Protein
Wilms tumor, also known as nephroblastoma, is a type of cancer that develops in the kidneys. It mostly affects children younger than 5 years old, though it can sometimes occur in older children and adults. Wilms tumor is the most common type of kidney cancer seen in children.
Structure and Properties of WT1 Protein
The Wilms tumor 1 (WT1) gene provides instructions for making a protein called Wilms tumor protein 1 or WT1. This protein plays a crucial role in the development of kidneys, gonads and other embryonic tissues. WT1 protein contains four zinc fingers that allow it to bind to specific regions of DNA and regulate the expression of target genes. It is comprised of 352-54 amino acids with a proline-glutamine rich N-terminal domain followed by four zinc finger motifs at the C-terminal end required for specific DNA binding. The protein contains potential phosphorylation and glycosylation sites that influence its function and stability. Alternative splicing of WT1 mRNA produces different protein isoforms with variable functions.
Role in Kidney Development
During kidney development, Wilms Tumor Protein is expressed in the ureteric bud to induce nephron progenitor cells and regulate their proliferation. It maintains the integrity of nephron progenitor cells to allow continuous generation of nephrons. WT1 is also important for mesenchymal-epithelial transition during glomerulus formation. In the absence of functional WT1, the metanephric mesenchyme fails to condense and undergo epithelial differentiation leading to impaired nephrogenesis. Thus, WT1 plays a crucial role in induction and maintenance of nephron progenitor cells to facilitate regular growth and patterning of the developing kidney.
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