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Transcript
Award ID:
RP100329
Project Title:
Next Generation Genomic Sequence Identification of the 19q Familial
Wilms Tumor Predisposition Gene
Award Mechanism:
Individual Investigator
Principal Investigator:
Huff, Vicki
Entity:
The University of Texas M.D. Anderson Cancer Center
Lay Summary:
Studying families who are genetically predisposed to cancer is a powerful approach for
identifying cancer genes. We have localized a familial predisposition gene for one cancer,
Wilms tumor (WT), a childhood cancer of the kidney, to chromosome 19. This result
implies that every affected person within one of these WT families carries the same DNA
alteration that predisposes them to cancer. To identify this genetic change, we will
sequence the DNA from the most distantly related affected members of five WT families
using the latest sequencing technologies. The significance of DNA changes shared by
family members will be further assessed in other WT families, in tumors from WT
patients who have no family history of WT, and in our mouse models for WT. From this
work we will identify a new gene that plays an important role in WT development. Other
"WT" genes are now known to also play important roles in embryogenesis and in the
development of other types of cancers. Thus, the identification of the chromosome 19 WT
predisposition gene will not only be a leap forward in understanding cancer
predisposition, but will likely also accelerate work in cancer genetics in general, identify
new therapeutic targets for cancer treatment, and provide insight into new approaches
for cancer prevention. As one of the first projects to sequence a region of the human
genome implicated in cancer predisposition, this work will also serve as a model for
identifying other human disease genes using large-scale genomic sequencing.