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

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' tu...

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Bibliographic Details
Published inOncogene Vol. 7; no. 4; p. 763
Main Authors Brown, K W, Watson, J E, Poirier, V, Mott, M G, Berry, P J, Maitland, N J
Format Journal Article
LanguageEnglish
Published England 01.04.1992
Subjects
Abnormalities, Multiple - genetics
Amino Acid Sequence
Aniridia - genetics
Base Sequence
Blotting, Southern
Chromosome Deletion
Chromosomes, Human, Pair 11
DNA, Neoplasm - genetics
DNA-Binding Proteins - genetics
Genes, Tumor Suppressor
Humans
Molecular Sequence Data
Oligodeoxyribonucleotides - chemistry
Polymerase Chain Reaction
RNA, Neoplasm - genetics
Syndrome
Urogenital Abnormalities
Wilms Tumor - genetics
WT1 Proteins
Zinc Fingers
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Summary: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.
ISSN:0950-9232
1476-5594