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

Article Abstract:

Wilms tumor is a nephroblastoma, or tumor of the kidney, which occurs in young children. Wilms tumor can be inherited and is associated with a specific chromosomal abnormality, the deletion of a particular region on chromosome 11. Other genitourinary abnormalities are also associated with this region of chromosome 11. The gene thought to be involved in Wilms tumors was identified using the molecular biological technique known as chromosome jumping. Chromosomes are structures composed of deoxyribose nucleic acid (DNA), which code for genes, and contain the information that directs molecules involved in the processes of life. Using probes against DNA sequences which are commonly found in genes that are expressed, several genes from this area on chromosome 11 were isolated. Using this technique of jumping the chromosome, large amounts of DNA sequence that do not contain sequences of interest can be by-passed. The protein that is coded by the gene thought to be involved in Wilms tumors contains sequences that are known as zinc fingers, a structural unit which binds the chemical zinc. Zinc fingers are present in proteins known as transcription factors. Transcription factors bind to DNA, and control the expression of various proteins coded for by the genes. It is not certain that the isolated gene causes Wilms tumors. More than one gene could exist in the chromosomal region that is altered in these tumors. The gene that was isolated was surprisingly not absent or rearranged in 63 out of 65 Wilms tumor samples examined. This isolated gene can also be used to study the embryonic development of kidneys, as Wilms tumors consist of undifferentiated or immature tissue. (Consumer Summary produced by Reliance Medical Information, Inc.)

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A gene for Wilms tumour?

Article Abstract:

Wilms tumor is a nephroblastoma, or tumor of the kidney, which occurs in young children. Wilms tumor can be inherited and is associated with a specific chromosomal abnormality, the deletion of a particular region on chromosome 11. Employing the same techniques used to isolate genes involved in other inherited diseases, such as Duchenne muscular dystrophy, X-linked chronic granulomatous disease, and retinoblastoma, a gene has been isolated which is thought to cause Wilms tumor. The protein which is coded for by the isolated gene contains sequences that are known as zinc fingers, a structural unit which binds the element zinc. Zinc fingers are present in proteins known as transcription factors. Transcription factors bind to deoxyribose nucleic acid (DNA) and control the expression of various proteins coded for by the genes. It is not certain that this isolated gene causes Wilms tumors; more than one gene could exist in the chromosomal region that is altered in Wilms tumors. However, the gene that has been isolated was surprisingly not absent or rearranged in 63 out of 65 Wilms tumor samples examined. Other researchers have also isolated the same gene. In some cases the inheritance of Wilms tumor is not associated with genes on chromosome 11. Wilms tumor formation may be a multistep process involving many genes. Even if other genes are involved, the isolation of this gene can lead to the isolation of the other genes. The gene can also be used to study the embryonic development of kidneys, as Wilms tumors consist of undifferentiated or immature tissue. (Consumer Summary produced by Reliance Medical Information, Inc.)

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The candidate Wilms' tumour gene is involved in genitourinary development

Article Abstract:

Wilms' tumor, a cancer of the kidneys, occurs during embryonic development and is associated with abnormalities of the urinary tract and the genitalia. Wilms' tumor is thought to arise from the loss of the function of a gene which suppresses tumor development. The chromosomes from individuals with WAGR syndrome, comprising Wilms' tumor, aniridia (absence of the iris of the eye), genitourinary abnormalities and mental retardation, have been analyzed and the gene that is deleted in individuals with Wilms' tumor was located at position 11p13. The defective gene in Wilms' tumor was then identified and analyzed. The gene encodes a protein that is similar to other proteins that bind to DNA known as zinc-finger proteins. This protein is thought to be a transcription factor, a protein that binds to DNA and regulates the transcription of other genes. The messenger RNA from various tissues of embryos and from Wilms' tumor was analyzed for the expression of this gene. The gene was expressed in certain areas of the developing kidney, in areas of the developing genitalia and in the Wilms' tumors. This suggests that the identified gene is indeed a gene that is defective in Wilms' tumor. The genital abnormalities that are associated with Wilms' tumors are due to changes in the genes that are normally expressed in the genitalia and are involved in the normal development of the kidney and parts of the genitalia. (Consumer Summary produced by Reliance Medical Information, Inc.)

Growth, Identification and classification, Genetic transcription, Transcription (Genetics), Genitourinary organs, Urogenital system

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