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

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Publication Type Journal Article
School or College College of Science; School of Medicine
Department Oncological Sciences; Human Genetics; Pathology; Pediatrics; Biology
Program Institute of Human Genetics; Howard Hughes Medical Institute (HHMI)
Creator Hansen, Mark S.; Coffin, Cheryl M.; Capecchi, Mario R.
Other Author Keller, Charles
Title Pax3:Fkhr interferes with embryonic Pax3 and Pax7 function: implications for alveolar rhabdomyosarcoma cell of origin.
Date 2004-11-01
Description 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.
Type Text
Publisher Cold Spring Harbor Lab Press
Volume 18
Issue 21
First Page 2608
Last Page 2613
Subject Neuroprogenitor; Embryogenesis
Subject MESH DNA-Binding Proteins; Gene Expression Regulation, Developmental; Homeodomain Proteins; Rhabdomyosarcoma, Alveolar; Transcription Factors
Language eng
Bibliographic Citation Keller C, Hansen MS, Coffin CM, Capecchi MR. Pax3:Fkhr interferes with embryonic Pax3 and Pax7 function: implications for alveolar rhabdomyosarcoma cell of origin.Genes Dev. 2004 Nov 1;18(21):2608-13. Retrieved on September 6, 2006 from http://www.genesdev.org/cgi/content/full/18/21/2608.
Rights Management Copyright 2004 Cold Spring Harbor Laboratory Press. All Rights Reserved.
Format Medium application/pdf
Identifier ir-main&CISOPTR=377
ARK ark:/87278/s6gt65jn
Setname ir_uspace
Date Created 2012-06-13
Date Modified 2012-06-13
ID 704780
Reference URL https://collections.lib.utah.edu/ark:/87278/s6gt65jn
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