This Article Full Text Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Keller, C. Articles by Capecchi, M. R. Search for Related Content PubMed PubMed Citation Articles by Keller, C. Articles by Capecchi, M. R. Related Collections Tumor Biology Tumor Biology: Model Organisms and Imaging

New Genetic Tactics to Model Alveolar Rhabdomyosarcoma in the Mouse

¹ Department of Cellular and Structural Biology, Children's Cancer Research Institute, The University of Texas Health Science Center, San Antonio, Texas and ² Howard Hughes Medical Institute and Department of Human Genetics, University of Utah, School of Medicine, Salt Lake City, Utah

Requests for reprints: Howard Hughes Medical Institute and Department of Human Genetics, University of Utah, School of Medicine, Salt Lake City, UT 84112-5331. Phone: 801-581-7096; Fax: 801-585-3425; E-mail: mario.capecchi{at}genetics.utah.edu.

Using conditional knock-in and knock-out techniques, we designed a mouse model of the childhood muscle cancer alveolar rhabdomyosarcoma (ARMS) that is driven by the chromosomal translocation product, Pax3:Fkhr. Tumors that closely recapitulate the spectrum of molecular markers and histology seen in human ARMS are exclusively produced in this model. Unexpectedly, expression of Pax3:Fkhr in muscle satellite cells did not produce tumors, but it did in differentiating myofibers. Expression of Pax3:Fkhr in muscle is necessary but not sufficient to initiate tumorigenesis at high frequency. This model offers new insight into the roots of alveolar rhabdomyosarcoma and illustrates the utility of Cre-loxP technology for studying otherwise inaccessible cancers in the mouse.

This article has been cited by other articles:

				A. J.F. Lazar, P. Das, D. Tuvin, B. Korchin, Q. Zhu, Z. Jin, C. L. Warneke, P. S. Zhang, V. Hernandez, D. Lopez-Terrada, et al. Angiogenesis-Promoting Gene Patterns in Alveolar Soft Part Sarcoma Clin. Cancer Res., December 15, 2007; 13(24): 7314 - 7321. [Abstract] [Full Text] [PDF]