Wilms tumor genes, also known as WT genes, are a group of genes that contribute to the development of Wilms tumor, the most common form of kidney cancer in children. Wilms tumor typically affects children between the ages of 2 and 5 years old.
These genes are primarily involved in regulating the growth and development of kidney cells during embryonic development. They play a crucial role in ensuring the normal proliferation and differentiation of kidney tissues, allowing them to mature into fully functional organs.
Mutations or alterations in these genes can disrupt this normal growth and development, leading to the formation of Wilms tumor. The two primary genes that are most frequently affected in Wilms tumor are WT1 and WT2, although other WT genes may also be involved in its development.
The WT1 gene, located on the short arm of chromosome 11, provides instructions for making a transcription factor protein that helps control the activity of other genes. Mutations in WT1 can impair the regulation of other genes involved in kidney development and lead to uncontrolled cell growth.
The WT2 gene, also known as WTX or FAM123B, is thought to act as a tumor suppressor gene, helping to prevent the formation and growth of tumors. Mutations in this gene can disrupt its tumor-suppressing function, contributing to the development of Wilms tumor.
Understanding the function and role of Wilms tumor genes is crucial for diagnosing and treating Wilms tumor in affected children. Genetic testing can help identify mutations in these genes, which can inform prognosis and guide treatment decisions.
