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RESEARCH COMMUNICATION
1 Division of Pediatric Hematology-Oncology, Department of Pediatrics, 2 Division of Pediatric Pathology, Department of Pathology, and 3 Howard Hughes Medical Institute and Department of Human Genetics, University of Utah, Salt Lake City, Utah 84112, USA
To investigate the role of the translocation-associated gene Pax3:Fkhr in alveolar rhabdomyosarcomas, we generated a Cre-mediated conditional knock-in of Pax3:Fkhr into the mouse Pax3 locus. Exploring embryonic tumor cell origins, we replaced a Pax3 allele with Pax3:Fkhr throughout its expression domain, causing dominant-negative effects on Pax3 and paradoxical activation of the Pax3 target gene, c-Met. Ectopic neuroprogenitor cell proliferation also occurs. In contrast, activation later in embryogenesis in cells that express Pax7 results in viable animals with a postnatal growth defect and a moderately decreased Pax7+ muscle satellite cell pool, phenocopying Pax7 deficiency but remarkably not leading to tumors.
[Keywords: Alveolar rhabdomyosarcoma; Pax3:Fkhr; Pax7; FoxO1A; chromosomal translocation; satellite cell]
Received July 26, 2004; revised version accepted September 3, 2004.
Article and publication are at http://www.genesdev.org/cgi/doi/10.1101/gad.1243904.
4 Corresponding author.
E-MAIL mario.capecchi{at}genetics.utah.edu; FAX (801) 585-3425.
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