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Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine [R. J. D., C. M. D., F. G. B.], and Division of Human Genetics and Molecular Biology, The Children's Hospital of Philadelphia [J. A. B.], Philadelphia, Pennsylvania 19104, and the Department of Pathology, University of Virginia, Charlottesville, Virginia 22908 [M. A. L.]
Although the t(2;13)(q35;q14) translocation has been found in most cases of the pediatric cancer alveolar rhabdomyosarcoma, several cases have been reported with a variant t(1;13)(p36;q14) translocation. Our findings indicate that this t(1;13) rearranges PAX7 on chromosome 1 and fuses it to FKHR on chromosome 13. This fusion results in a chimeric transcript consisting of 5' PAX7 and 3' FKHR regions, which is similar to the 5' PAX3-3' FKHR transcript formed by the t(2;13). The 5' PAX3 and PAX7 regions encode related DNA binding domains, and therefore we postulate that these translocations create similar chimeric transcription factors that alter expression of a common group of target genes.
1 This work was supported by National Cancer Institute Grant CA47983 (to F. G. B. and J. A. B.) and the University of Pennsylvania Foundation (to F. G. B.)
2 To whom requests for reprints should be addressed, at Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, 205 John Morgan Building, Philadelphia, PA 19104.
Received 3/21/94. Accepted 4/21/94.
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|
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|
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|
|
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|
|
|
|
|
|
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|
|
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|
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|
|
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