Nucleic Acids Research, 1994, Vol. 22, No. 22 4574-4582
© 1994
MOLECULAR BIOLOGY |
Molecular cloning and characterization of a human PAX-7 cDNA expressed in normal and neoplastic myocytes
University of Zürich, Department of Pediatrics, Division of Clinical Chemistry Steinwiesstrasse 75, 8032 Zürich, Switzerland 1Research Institute of Molecular Pathology Dr. Bohr-Gasse 7, 1030 Vienna, Austria
*To whom correspondence should be addressed
Received August 25, 1994. Revised October 11, 1994. Accepted October 11, 1994.
The myogenic basic helix-loop-helix proteins are essential components of the regulatory network controlling vertebrate myogenesis. However, determined myoblasts appear in the limb buds which do not initially express any member of this transcription factor family. In a search for potential novel regulators of myogenesis, a human PAX-7 cDNA was isolated from primary myoblasts. Analysis of the DNA-binding properties of the Pax-7 paired domain revealed that it binds DNA in a sequence-specific manner indistinguishable from that of the paralogous Pax-3 protein. Each of the two proteins also binds to palindromic homeodomainbinding sites by cooperative dimerization. Both Pax-3 and Pax-7, which are known to partially overlap in their expression during development, can also efficiently form heterodimers on these sites and stimulate reporter gene transcription in transient transfection experiments which, in the case of Pax-7, is dependent on the transactivation function encoded by the C-terminal sequences. Thus, the formation of heterodimers might have important consequences for target gene recognition and regulation during development. PAX-7 was found to be weakly expressed in normal human myoblasts, while PAX-3 could not be detected in these cells at all. However, transcripts for either PAX-3 and/or PAX-7 were expressed at elevated levels in tumorigenic rhabdomyosarcoma cell lines. Hence, overexpression of these PAX genes may be involved in the genesis of myogenic tumors.
CiteULike 
Connotea 
Del.icio.us What's this?
This article has been cited by other articles:
|
|
 |
|
  M. Wachtel, M. Dettling, E. Koscielniak, S. Stegmaier, J. Treuner, K. Simon-Klingenstein, P. Buhlmann, F. K. Niggli, and B. W. Schafer Gene Expression Signatures Identify Rhabdomyosarcoma Subtypes and Detect a Novel t(2;2)(q35;p23) Translocation Fusing PAX3 to NCOA1 Cancer Res., August 15, 2004; 64(16): 5539 - 5545. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 S. Apuzzo, A. Abdelhakim, A. S. Fortin, and P. Gros Cross-talk between the Paired Domain and the Homeodomain of Pax3: DNA BINDING BY EACH DOMAIN CAUSES A STRUCTURAL CHANGE IN THE OTHER DOMAIN, SUPPORTING INTERDEPENDENCE FOR DNA BINDING J. Biol. Chem., August 6, 2004; 279(32): 33601 - 33612. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. J. Anderson, G. D. Shelton, W. K. Cavenee, and K. C. Arden Embryonic expression of the tumor-associated PAX3-FKHR fusion protein interferes with the developmental functions of Pax3 PNAS, February 13, 2001; 98(4): 1589 - 1594. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
F. A. Scholl, J. Kamarashev, O. V. Murmann, R. Geertsen, R. Dummer, and B. W. Schäfer PAX3 Is Expressed in Human Melanomas and Contributes to Tumor Cell Survival Cancer Res., February 1, 2001; 61(3): 823 - 826. [Abstract] [Full Text]
|
|
|
|
|
|
K. Ayyanathan, W. J. Fredericks, C. Berking, M. Herlyn, C. Balakrishnan, E. Gunther, and F. J. Rauscher III Hormone-dependent Tumor Regression in Vivo by an Inducible Transcriptional Repressor Directed at the PAX3-FKHR Oncogene Cancer Res., October 1, 2000; 60(20): 5803 - 5814. [Abstract] [Full Text]
|
|
|
|
 |
|
 W. J. Fredericks, K. Ayyanathan, M. Herlyn, J. R. Friedman, and F. J. Rauscher III An Engineered PAX3-KRAB Transcriptional Repressor Inhibits the Malignant Phenotype of Alveolar Rhabdomyosarcoma Cells Harboring the Endogenous PAX3-FKHR Oncogene Mol. Cell. Biol., July 15, 2000; 20(14): 5019 - 5031. [Abstract] [Full Text]
|
|
|
|
|
|
J. Khan, M. L. Bittner, L. H. Saal, U. Teichmann, D. O. Azorsa, G. C. Gooden, W. J. Pavan, J. M. Trent, and P. S. Meltzer cDNA microarrays detect activation of a myogenic transcription program by the PAX3-FKHR fusion oncogene PNAS, November 9, 1999; 96(23): 13264 - 13269. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
F. G. Barr, J. C. Fitzgerald, J. P. Ginsberg, M. L. Vanella, R. J. Davis, and J. L. Bennicelli Predominant Expression of Alternative PAX3 and PAX7 Forms in Myogenic and Neural Tumor Cell Lines Cancer Res., November 1, 1999; 59(21): 5443 - 5448. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. A. Epstein, B. Song, M. Lakkis, and C. Wang Tumor-Specific PAX3-FKHR Transcription Factor, but Not PAX3, Activates the Platelet-Derived Growth Factor Alpha Receptor Mol. Cell. Biol., July 1, 1998; 18(7): 4118 - 4130. [Abstract] [Full Text]
|
|
|
|
|
|
M. Bernasconi, A. Remppis, W. J. Fredericks, F. J. Rauscher III, and B. W. Schafer Induction of apoptosis in rhabdomyosarcoma cells through down-regulation of PAX proteins PNAS, November 12, 1996; 93(23): 13164 - 13169. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S. Nornes, I. Mikkola, S. Krauss, M. Delghandi, M. Perander, and T. Johansen Zebrafish Pax9 Encodes Two Proteins with Distinct C-terminal Transactivating Domains of Different Potency Negatively Regulated by Adjacent N-terminal Sequences J. Biol. Chem., October 25, 1996; 271(43): 26914 - 26923. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. A. Epstein, P. Lam, L. Jepeal, R. L. Maas, and D. N. Shapiro Pax3 Inhibits Myogenic Differentiation of Cultured Myoblast Cells J. Biol. Chem., May 19, 1995; 270(20): 11719 - 11722. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. G. Ridgeway and I. S. Skerjanc Pax3 Is Essential for Skeletal Myogenesis and the Expression of Six1 and Eya2 J. Biol. Chem., May 25, 2001; 276(22): 19033 - 19039. [Abstract] [Full Text] [PDF]
|
|