A critical mutation in both WT1 alleles is not sufficient to cause Wilms' tumor

The WT1 gene is a tumor suppresser gene for Wilms' tumor (WT). Inactivation of both alleles has been proposed as the cause of WT. We encountered a patient with Denys-Drash syndrome associated with WT whose WT1 gene had a homozygous point mutation not only in WT but also in renal tissue adjacent...

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Bibliographic Details
Published in:FEBS letters 1995-02, Vol.360 (1), p.26-28
Main Authors: Kikuchi, Haruhito, Akasaka, Yoshikiyo, Kurosawa, Yoshihiro, Yoneyama, Hiroshi, Kato, Shingo, Hata, Jun-ichi
Format: Article
Language:English
Subjects:
Alleles
Base Sequence
Blotting, Southern
DNA Primers
Genes, Wilms Tumor
Humans
Infant
Molecular Sequence Data
Mutation
Polymerase Chain Reaction
Wilms Tumor - etiology
Wilms Tumor - genetics
Wilms' tumor
WT1 gene
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Summary:The WT1 gene is a tumor suppresser gene for Wilms' tumor (WT). Inactivation of both alleles has been proposed as the cause of WT. We encountered a patient with Denys-Drash syndrome associated with WT whose WT1 gene had a homozygous point mutation not only in WT but also in renal tissue adjacent to the WT and in the germline. These findings indicate that factor(s) other than the loss of WT1 are required for WT to develop.
ISSN:0014-5793
1873-3468
DOI:10.1016/0014-5793(95)00071-G