An internal deletion within an 11p13 zinc finger gene contributes to the development of Wilms' tumor

doi:10.1016/0092-8674(90)90690-G

Cell

Volume 61, Issue 7, 29 June 1990, Pages 1257-1269

https://doi.org/10.1016/0092-8674(90)90690-G Get rights and content

Abstract

We have recently described the isolation of a candidate for the Wilms' tumor susceptibility gene mapping to band p13 of human chromosome 11. This gene, primarily expressed in fetal kidney, appears to encode a DNA binding protein. We now describe a sporadic, unilateral Wilms' tumor in which one allele of this gene contains a 25 bp deletion spanning an exon-intron junction and leading to aberrant mRNA splicing and loss of one of the four zinc finger consensus domains in the protein. The mutation is absent in the affected individual's germline, consistent with the somatic inactivation of a tumor suppressor gene. In addition to this intragenic deletion affecting one allele, loss of heterozygosity at loci along the entire chromosome 11 points to an earlier chromosomal nondisjunction and reduplication. We conclude that inactivation of this gene, which we call WT1, is part of a series of events leading to the development of Wilms' tumor.

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Cell

ArticleAn internal deletion within an 11p13 zinc finger gene contributes to the development of Wilms' tumor

Abstract

Cell

J. Urol.

Cell

Cell

Biochem. Biophys. Res. Commun.

Cell

Cell

Genomics

Cell

J. Biol. Chem.

Genomics

Cell

Cell

J. Pediatr.

Purification of mouse immunoglobin heavy-chain messenger RNAs from total myeloma tumor RNA

Eur. J. Biochem.

Chromosome 17 deletions and p53 gene mutations in colorectal carcinomas

Science

Patient with 13 chromosome deletion: evidence that the retinoblastoma gene is a recessive cancer gene

Science

Structure and expression of the murine retinoblastoma gene and characterization of its encoded protein

Human retinoblastoma susceptibility gene: genomic organization and analysis of heterozygous intragenic deletion mutants

Expression of recessive alleles by chromosomal mechanisms in retinoblastoma

Nature

Genetic origin of mutations predisposing to retinoblastoma

Science

Altering DNA-binding specificity of GAL4 requires sequences adjacent to the zinc finger

Nature

Protein encoded by v-erbA functions as a thyroid-hormone receptor antagonist

Nature

Homozygosity of chromosome 13 in retinoblastoma

N. Engl. J. Med.

Molecular detection of deletions involving band q14 of chromosome 13 in retinoblastomas

Identification of germline and somatic mutations affecting the retinoblastoma gene

Science

Mutations in the RB1 gene and their effects on transcription

Mol. Cell. Biol.

Somatic deletion and duplication of genes on chromosome 11 in Wilms' tumours

Nature

Aniridia-Wilms' tumor association: evidence for specific deletion of 11p13

Cytogenet. Cell Genet.

A human DNA segment with properties of the gene that predisposes to retinoblastoma and osteosarcoma

Nature

Deletions of a DNA sequence in retinoblastomas and mesenchymal tumors: organization of the sequence and its encoded protein

Structural evidence for the authenticity of the human retinoblastoma susceptibility gene

Science

Homozygous deletion in Wilms' tumours of a zinc-finger gene identified by chromosome jumping

Nature

Constitutional and somatic mutations of chromosome 11p in Wilms' tumor

Infrequent genomic rearrangement and normal expression of the putative RB1 gene in retinoblastoma tumors

Mol. Cell. Biol.

Isolation of high molecular weight DNA from mammalian cells

Eur. J. Biochem.

Familial predisposition to Wilms' tumour does not map to the short arm of chromosome 11

Nature

Abnormalities in structure and expression of the human retinoblastoma gene in SCLC

Science

Tumor-specific loss of 11p15.5 alleles in del11p13 Wilms tumor and in familial adrenocortical carcinoma

Article
An internal deletion within an 11p13 zinc finger gene contributes to the development of Wilms' tumor