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[Cancer Research 53, 5108-5112, November 1, 1993]
© 1993 American Association for Cancer Research
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Fusion of PAX3 to a Member of the Forkhead Family of Transcription Factors in Human Alveolar Rhabdomyosarcoma1

David N. Shapiro2, Jack E. Sublett, Baitao Li, James R. Downing and Clayton W. Naeve

Departments of Experimental Oncology [D. N. S., J. E. S., B. L.], Hematology/Oncology [D. N. S.], Pathology [J. R. D.], Tumor Cell Biology [J. R. D.], and Virology and Molecular Biology [C. W. N.], St. Jude Children's Research Hospital, Memphis, Tennessee 38105, and Departments of Pediatrics [D. N. S.] and Pathology [J. R. D., C. W. N.], University of Tennessee College of Medicine, Memphis, Tennessee 38163

Alveolar rhabdomyosarcoma, a malignant tumor of skeletal muscle, is characterized by a chromosomal translocation, t(2;13)(q35;q14). This translocation is associated with a structural rearrangement of the gene encoding PAX3, a presumed transcriptional regulator expressed exclusively during embryogenesis. The breakpoint results in a fusion between PAX3 and a gene provisionally named ALV, a novel member of the forkhead family of transcription factors. In PAX3-ALV, the structural integrity of both PAX3 DNA-binding regions, the paired box and homeodomain, are retained while the putative transcriptional activation domain of PAX3 is replaced by the bisected forkhead DNA-binding domain of ALV. Formation of chimeric transcription factors has now been implicated in diverse human tumors of myogenic, hematopoietic, neuroectodermal, and adipocytic origin, suggesting that transcriptional deregulation is a common mechanism of tumorigenesis.

1 Supported in part by Grant CA-23099 and Cancer Center CORE Grant CA-21765 from the NIH and by the American Lebanese Syrian Associated Charities (ALSAC).

2 To whom requests for reprints should be addressed, at the Department of Experimental Oncology, St. Jude Children's Research Hospital, 332 North Lauderdale, Memphis, TN 38105.

Received 9/29/93. Accepted 10/ 1/93.




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