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Characterization of the chromosome 22 region altered in malignant rhabdoid tumors

Objective



Research objectives and content
Rhabdoid tumors (MRT) are highly malignant tumors occuring in young children. They can occur in renal or extra-renal localization and are frequently difficult to diagnose. Cytogenetics and molecular biology have indicated that MRT recurrently demonstrate deletion of the proximal part of chromosome 22. This strongly suggests that oncogenesis of these tumors is linked to the inactivation of a presently unknown tumor suppressor gene localized on chromosome 22. This project is aimed at the precise characterization of an MRT cell line, established in the laboratory, which demonstrates an homozygous deletion of chromosome 22. This, together with the analysis of additional MRT will be the basis for a positional cloning approach of the MRT tumor suppressor gene, including construction of cosmid contigs, search for transcription unit and screen for point mutations. Isolation of this new gene would enable to test its alteration in other tumor types characterized by alteration of chromosome 22 and should be important for the development of new diagnostic strategies for MRT. Training content (objective,benefit and expected impact)
- Theoretical and practical training in oncogenesis - Positional cloning strategies - Analysis of gene alteration - Human cancer, pediatric tumors - Interaction between basic science and clinics
Links with industry/industrial relevance(22)
Development of diagnostic tools for small round cell tumors including Ewing tumor, neuroblastoma and MRT

Funding Scheme

RGI - Research grants (individual fellowships)

Coordinator

Institut Curie
Address
26,Rue D'ulm
75231 Paris
France

Participants (1)

Not available
Netherlands