What does WT1 stand for?

Clinical significance Mutations of Wilms’ tumor suppressor gene1 (WT1) are associated with embryonic malignancy of the kidney, affecting around 1-9 in 100,000 infants. It occurs in both sporadic and hereditary forms.

Is WT1 a tumor suppressor gene?

In human hematopoietic cells, WT1 appears to behave as a tumor suppressor gene as the overexpression of WT1 in early human bone marrow cells leads to growth arrest and reduced colony formation. This effect is due to the zinc fingers, as deletion of this domain abolishes the effect.

What is WT1 mutation?

WT1 gene mutations that cause Wilms tumor lead to a WT1 protein with a decreased ability to bind to DNA. As a result, the protein cannot regulate gene activity, leading to uncontrolled growth and division of cells in the kidney and allowing tumor development.

What WT1 positive?

WT1-positive tumors included tumor of the stomach, prostate, and biliary and urinary systems, and malignant melanomas. A majority of the positive cases showed diffuse or granular staining in the cytoplasm, whereas ovarian tumors and desmoplastic small round cell tumors frequently showed nuclear staining.

What is another name for Nephroblastoma?

Wilms tumor (also called Wilms’ tumor or nephroblastoma) is a type of childhood cancer that starts in the kidneys. It is the most common type of kidney cancer in children. About 9 of 10 kidney cancers in children are Wilms tumors.

What is gata3 positive?

Gene expression profiling has shown that GATA-3 is highly expressed in the Luminal A subtype of breast cancer. A recent study found GATA-3 to be associated with favorable breast cancer pathologic features, including negative lymph node and positive estrogen receptor (ER) status.

What happens if the p53 protein is mutated?

Mutations (changes) in the p53 gene may cause cancer cells to grow and spread in the body. These changes have been found in a genetic condition called Li-Fraumeni syndrome and in many types of cancer. The p53 gene is a type of tumor suppressor gene.

What is the function of the WT1 gene?

The WT1 gene provides instructions for making a protein that is necessary for the development of the kidneys and gonads (ovaries in females and testes in males) before birth. After birth, WT1 protein activity is limited to a structure known as the glomerulus, which filters blood through the kidneys.

What kind of tumor is WT1 associated with?

WT1 is a tumor suppressor gene associated with the development of Wilms’ Tumor, from which it was named. Mutations in exon 7 and 9 of WT1 have been recurrently identified in acute myeloid leukemia and associated with poorer prognosis and chemotherapy resistance. WT1 (WT1 Transcription Factor) is a Protein Coding gene.

What happens if you inactivate the WT1 protein?

Inactivation of WT1 causes Wilms tumour, and Denys-Drash syndrome (DDS), leading to nephropathy and genital abnormalities. The WT1 protein has been found to bind a host of cellular factors, e.g. p53, a known tumor suppressor. Despite the name, WT1 mutation is found in only about 5-10% of Wilms Tumor cases.

What does the WT1 protein do after birth?

After birth, WT1 protein activity is limited to a structure known as the glomerulus, which filters blood through the kidneys. The WT1 protein plays a role in cell growth, the process by which cells mature to perform specific functions (differentiation), and the self-destruction of cells (apoptosis).