A consistent t(2;13) (q35;q14) translocation in alveolar RMS results in fusion between PAX3 or PAX7 genes with the FKHR factors. Using reverse transcription (RT)-PCR and fluorescence in situ hybridization (FISH) technique, we will investigate the presence of PAX3 (PAX7)/FKHR transcripts in a large number of alveolar RMS. Embryonal RMS on the other hand exhibits a reproducible loss of chromosomal material on chromosome 11p15.5. We have used oligonucleotide repeats at the tyrosine hydroxylase (TH) locus and investigated loss of heterozygosity (LOH) in 11 RMS and 13 non-RMS sarcomas in children and young adults. We found consistent LOH at the TH locus in all but one (botryoid) RMS. However, LOH was also detected in 2 nerve sheath tumors and one fibrosarcoma. We would like to extend our studies to include a larger sample of RMS and non-RMS tumors. Our preliminary findings however suggest, that although the RT-PCR detected fusion transcripts are specific for alveolar RMS, LOH at the 11p15.5 locus is present not only in embryonal RMS, but other tumors as well.

Agency
National Institute of Health (NIH)
Institute
National Cancer Institute (NCI)
Type
Intramural Research (Z01)
Project #
1Z01SC009431-02
Application #
2464541
Study Section
Special Emphasis Panel (LP)
Project Start
Project End
Budget Start
Budget End
Support Year
2
Fiscal Year
1996
Total Cost
Indirect Cost
Name
National Cancer Institute Division of Clinical Sciences
Department
Type
DUNS #
City
State
Country
United States
Zip Code
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