Article
Antagonism of WT1 activity by protein self-association.
Proceedings of the National Academy of Sciences of the United States of America - 21 Nov 1995
Moffett P, Bruening W, Nakagama H, Bardeesy N, Housman D, Housman D E, Pelletier J
Abstract excerpt
Germline loss-of-function mutations at the Wilms tumor (WT) suppressor locus WT1 are associated with a predisposition to WTs and mild genital system anomalies. In contrast, germ-line missense mutations within the WT1 gene encoding the DNA-binding domain often yield a more severe phenotype consisting of WT, sexual ambiguity, and renal nephropathy. In this report, we demonstrate that the products of mutant alleles...
Topics
- Alleles
- DNA-Binding Proteins
- Gene Expression Regulation, Neoplastic
- Genes, Wilms Tumor
- Humans
- Macromolecular Substances
- Promoter Regions, Genetic
- Protein Binding
- Receptors, Retinoic Acid
- Recombinant Proteins