TY - JOUR
T1 - Linkage of familial Wilms' tumor predisposition to two-locus model for the etiology of familial tumors
AU - McDonald, J. Matthew
AU - Douglass, Edwin C.
AU - Fisher, Ross
AU - Geiser, Clementina F.
AU - Krill, Carl E.
AU - Strong, Louise C.
AU - Virshup, David
AU - Huff, Vicki
PY - 1998/4/1
Y1 - 1998/4/1
N2 - 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.
AB - 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.
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M3 - Article
C2 - 9537236
AN - SCOPUS:0032053822
SN - 0008-5472
VL - 58
SP - 1387
EP - 1390
JO - Cancer Research
JF - Cancer Research
IS - 7
ER -