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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. [7] [12] [13] [14] Despite the name, WT1 mutation is found in only about 5-10% of Wilms Tumor cases. [15]
Wilms' tumor or Wilms tumor, [3] also known as nephroblastoma, is a cancer of the kidneys that typically occurs in children (rarely in adults), [4] and occurs most commonly as a renal tumor in child patients. [5] [6] It is named after Max Wilms, the German surgeon (1867–1918) who first described it. [7]
WAGR syndrome (also known as WAGR complex, Wilms tumour-aniridia syndrome, aniridia-Wilms tumour syndrome) is a rare genetic syndrome in which affected children are predisposed to develop Wilms' tumour (a tumour of the kidneys), aniridia (absence of the coloured part of the eye, the iris), genitourinary anomalies, and mental retardation. [1]
Pre-mRNA-splicing regulator WTAP is a protein that in humans is encoded by the WTAP gene. [5] [6] [7]The Wilms tumor suppressor gene WT1 appears to play a role in both transcriptional and posttranscriptional regulation of certain cellular genes.
The National Wilms Tumor Study Group (NWTS) is a cancer research cooperative group in the United States formed to study a type of kidney tumor that affects children called Wilms' tumor. In 2001, NWTS merged with several other pediatric oncology cooperative groups to create the Children's Oncology Group (COG). However, the NWTS is still active ...
In 2008, leukemia, usually acute lymphoblastic leukemia, was the most common cancer in children aged 1–14 in the U.S., followed by the central nervous system cancers, neuroblastoma, Wilms' tumor, and non-Hodgkin's lymphoma. [20] Childhood cancers increased 19% between 1975 and 1990, mainly due to an increased incidence in acute leukemia.