Pax3:Fkhr interferes with embryonic Pax3 and Pax7 function: Implications for alveolar rhabdomyosarcoma cell of origin

Charles Keller, Mark S. Hansen, Cheryl M. Coffin, Mario R. Capecchi

Research output: Contribution to journalArticle

156 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)2608-2613
Number of pages6
JournalGenes and Development
Issue number21
StatePublished - Nov 1 2004



  • Alveolar rhabdomyosarcoma
  • Chromosomal translocation
  • FoxO1A
  • Pax3:Fkhr
  • Pax7
  • Satellite cell

ASJC Scopus subject areas

  • Genetics
  • Developmental Biology

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