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Molecular and Cellular Biology Volume 18, 1998 - Issue 7
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Cell Growth and Development

Tumor-Specific PAX3-FKHR Transcription Factor, but Not PAX3, Activates the Platelet-Derived Growth Factor Alpha Receptor

Jonathan A. Epsteina Cardiovascular Division, Department of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania19104
,
Baoliang Songb Center for Molecular Biology of Oral Diseases, University of Illinois at Chicago, Chicago, Illinois60612
,
Maha Lakkisa Cardiovascular Division, Department of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania19104
&
Chiayeng Wangb Center for Molecular Biology of Oral Diseases, University of Illinois at Chicago, Chicago, Illinois60612Correspondence[email protected]
Pages 4118-4130 | Received 20 Oct 1997, Accepted 06 Apr 1998, Published online: 28 Mar 2023

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M Ebauer, M Wachtel, F K Niggli & B W Schäfer. (2007) Comparative expression profiling identifies an in vivo target gene signature with TFAP2B as a mediator of the survival function of PAX3/FKHR. Oncogene 26:51, pages 7267-7281.
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L Zhang & C Wang. (2006) Identification of a new class of PAX3-FKHR target promoters: a role of the Pax3 paired box DNA binding domain. Oncogene 26:11, pages 1595-1605.
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Huiping Zhu, Ned J Wicker, Kelly Volcik, Jing Zhang, Gary M Shaw, Edward J Lammer, Lucina Suarez, Mark Canfield & Richard H Finnell. (2004) Promoter haplotype combinations for the human PDGFRA gene are associated with risk of neural tube defects. Molecular Genetics and Metabolism 81:2, pages 127-132.
Crossref
Lei Zhang & Chiayeng Wang. (2003) PAX3-FKHR Transformation Increases 26 S Proteasome-dependent Degradation of p27Kip1, a Potential Role for Elevated Skp2 Expression. Journal of Biological Chemistry 278:1, pages 27-36.
Crossref
Paul H.L.J Joosten, Mascha Toepoel, Dirk van Oosterhout, Gijs B Afink & Everardus J.J van Zoelen. (2002) A regulating element essential for PDGFRA transcription is recognized by neural tube defect-associated PRX homeobox transcription factors. Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease 1588:3, pages 254-260.
Crossref
Peter Carlsson & Margit Mahlapuu. (2002) Forkhead Transcription Factors: Key Players in Development and Metabolism. Developmental Biology 250:1, pages 1-23.
Crossref
Thomas D Barber, Melisa C Barber, Oana Tomescu, Frederic G Barr, Steve Ruben & Thomas B Friedman. (2002) Identification of Target Genes Regulated by PAX3 and PAX3–FKHR in Embryogenesis and Alveolar Rhabdomyosarcoma. Genomics 79:3, pages 278-284.
Crossref
O'Neil Wiggan, Marc P. Fadel & Paul A. Hamel. (2002) Pax3 induces cell aggregation and regulates phenotypic mesenchymal-epithelial interconversion. Journal of Cell Science 115:3, pages 517-529.
Crossref
C.S.K. Mayanil, David George, Laura Freilich, Erik J. Miljan, Barbara Mania-Farnell, David G. McLone & Eric G. Bremer. (2001) Microarray Analysis Detects Novel Pax3 Downstream Target Genes. Journal of Biological Chemistry 276:52, pages 49299-49309.
Crossref
D. Ayalon, T. Glaser & H. Werner. (2001) Transcriptional regulation of IGF-I receptor gene expression by the PAX3–FKHR oncoprotein. Growth Hormone & IGF Research 11:5, pages 289-297.
Crossref
Frederic G Barr. (2001) Gene fusions involving PAX and FOX family members in alveolar rhabdomyosarcoma. Oncogene 20:40, pages 5736-5746.
Crossref
John Anderson, Alan Ramsay, Steve Gould & Kathryn Pritchard-Jones. (2001) PAX3-FKHR Induces Morphological Change and Enhances Cellular Proliferation and Invasion in Rhabdomyosarcoma. The American Journal of Pathology 159:3, pages 1089-1096.
Crossref
Paul H.L.J. Joosten, Mascha Toepoel, Edwin C.M. Mariman & Everardus J.J. Van Zoelen. (2001) Promoter haplotype combinations of the platelet-derived growth factor α-receptor gene predispose to human neural tube defects. Nature Genetics 27:2, pages 215-217.
Crossref
D Alan Underhill. (2000) Genetic and biochemical diversity in the Pax gene family . Biochemistry and Cell Biology 78:5, pages 629-638.
Crossref
Yun Cao & Chiayeng Wang. (2000) The COOH-terminal Transactivation Domain Plays a Key Role in Regulating the in Vitro and in Vivo Function of Pax3 Homeodomain. Journal of Biological Chemistry 275:13, pages 9854-9862.
Crossref
John Anderson, Anthony Gordon, Kathy Pritchard-Jones & Janet Shipley. (1999) Genes, chromosomes, and rhabdomyosarcoma. Genes, Chromosomes and Cancer 26:4, pages 275-285.
Crossref
Javed Khan, Michael L. Bittner, Lao H. Saal, Ulrike Teichmann, David O. Azorsa, Gerald C. Gooden, William J. Pavan, Jeffrey M. Trent & Paul S. Meltzer. (1999) cDNA microarrays detect activation of a myogenic transcription program by the PAX3-FKHR fusion oncogene . Proceedings of the National Academy of Sciences 96:23, pages 13264-13269.
Crossref
Pierre Aman. (1999) Fusion genes in solid tumors. Seminars in Cancer Biology 9:4, pages 303-318.
Crossref
Jeannette L. Bennicelli & Frederic G. Barr. (1999) Genetics and the biologic basis of sarcomas. Current Opinion in Oncology 11:4, pages 267.
Crossref
Jun Li, Kenneth C. Liu, Fuzi Jin, Min Min Lu & Jonathan A. Epstein. (1999) Transgenic rescue of congenital heart disease and spina bifida in Splotch mice . Development 126:11, pages 2495-2503.
Crossref

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