Inactivation of the remaining allele of the WT1 gene in a Wilms' tumour from a WAGR patient.

Abstract:

:A candidate gene (WT1) has recently been described for the 11p13 tumour-suppressor gene involved in the development of Wilms' tumour. This gene encodes a zinc finger protein which can bind to a specific DNA sequence. We have found a 226 base deletion in the mRNA from a unilateral Wilms' tumour, which would cause a frameshift that completely deletes the zinc finger domain. The tumour developed in a patient suffering from the WAGR syndrome, who had a constitutional 11p13 deletion, and so the 226 base deletion represents the inactivation of the remaining WT1 allele in the tumour. This provides further direct evidence that loss of function of WT1 is an essential step in the development of Wilms' tumour.

journal_name

Oncogene

journal_title

Oncogene

authors

Brown KW,Watson JE,Poirier V,Mott MG,Berry PJ,Maitland NJ

subject

Has Abstract

pub_date

1992-04-01 00:00:00

pages

763-8

issue

4

eissn

0950-9232

issn

1476-5594

journal_volume

7

pub_type

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