Pax3:Fkhr interferes with embryonic Pax3 and Pax7 function:: implications for alveolar rhabdomyosarcoma cell of origin
Pax3:Fkhr interferes with embryonic Pax3 and Pax7 function:: implications for alveolar rhabdomyosarcoma cell of origin
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DOI：
10.1101/gad.1243904
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发表时间：
2004-11-01
影响因子：
10.5
通讯作者：
Capecchi, MR
中科院分区：
文献类型：
--
作者：
Keller, C;Hansen, MS;Capecchi, MR
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.
