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The Hybrid PAX3-FKHR Fusion Protein of Alveolar Rhabdomyosarcoma Transforms Fibroblasts in Culture
Sabine Scheidler, William J. Fredericks, Frank J. Rauscher, Frederic G. Barr and Peter K. Vogt
Proceedings of the National Academy of Sciences of the United States of America
Vol. 93, No. 18 (Sep. 3, 1996), pp. 9805-9809
Published by: National Academy of Sciences
Stable URL: http://www.jstor.org/stable/39792
Page Count: 5
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Pediatric alveolar rhabdomyosarcoma is characterized by a chromosomal translocation that fuses parts of the PAX3 and FKHR genes. PAX3 codes for a transcriptional regulator that controls developmental programs, and FKHR codes for a forkhead-winged helix protein, also a likely transcription factor. The PAX3-FKHR fusion product retains the DNA binding domains of the PAX3 protein and the putative activator domain of the FKHR protein. The PAX3-FKHR protein has been shown to function as a transcriptional activator. Using the RCAS retroviral vector, we have introduced the PAX3-FKHR gene into chicken embryo fibroblasts. Expression of the PAX3-FKHR protein in these cells leads to transformation: the cells become enlarged, grow tightly packed and in multiple layers, and acquire the ability for anchorage-independent growth. This cellular transformation in vitro will facilitate studies on the mechanism of PAX3-FKHR-induced oncogenesis.
Proceedings of the National Academy of Sciences of the United States of America © 1996 National Academy of Sciences