Linkage of familial Wilms' tumor predisposition to chromosome 19 and a two-locus model for the etiology of familial tumors.

Published

Journal Article

Familial predisposition to Wilms' tumor (WT), a childhood kidney tumor, is inherited as an autosomal dominant trait. For most WT families studied, the 11p13 gene WT1 and genomic regions implicated in tumorigenesis in a subset of tumors can be ruled out as the site of the familial predisposition gene. Following a genome-wide genetic linkage scan, we have obtained strong evidence (log of the odds ratio = 4.0) in five families for an inherited WT predisposition gene (FWT2) at 19q13.3-q13.4. In addition, we observed loss of heterozygosity at 19q in tumors from individuals from two families in which 19q can be ruled out as the site of the inherited predisposing mutation. From these data, we hypothesize that alterations at two distinct loci are critical rate-limiting steps in the etiology of familial WTs.

Full Text

Duke Authors

Cited Authors

  • McDonald, JM; Douglass, EC; Fisher, R; Geiser, CF; Krill, CE; Strong, LC; Virshup, D; Huff, V

Published Date

  • April 1, 1998

Published In

Volume / Issue

  • 58 / 7

Start / End Page

  • 1387 - 1390

PubMed ID

  • 9537236

Pubmed Central ID

  • 9537236

International Standard Serial Number (ISSN)

  • 0008-5472

Language

  • eng

Conference Location

  • United States