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Pax7  -  paired box 7

Mus musculus

Synonyms: Paired box protein Pax-7, Pax-7
 
 
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Disease relevance of Pax7

 

High impact information on Pax7

  • Cell culture and electron microscopic analysis revealed a complete absence of satellite cells in Pax7(-/-) skeletal muscle [4].
  • Importantly, stem cells from Pax7(-/-) muscle displayed almost a 10-fold increase in their ability to form hematopoietic colonies [4].
  • The paired box transcription factor Pax7 was isolated by representational difference analysis as a gene specifically expressed in cultured satellite cell-derived myoblasts [4].
  • Pax7 is required for the specification of myogenic satellite cells [4].
  • Pax3/Pax7 mark a novel population of primitive myogenic cells during development [5].
 

Biological context of Pax7

 

Anatomical context of Pax7

  • The growth and repair of skeletal muscle after birth depends on satellite cells that are characterized by the expression of Pax7 [9].
  • Pax3 and Pax7 have distinct and overlapping functions in adult muscle progenitor cells [9].
  • Pax3 and Pax7 are expressed in commissural neurons and restrict ventral neuronal identity in the spinal cord [10].
  • Whilst initial Pax7-specification of the mesencephalon is well-established, a role in regulating polarity within the maturing mouse superior colliculus is yet to be defined, although already detailed for the chick tectum [11].
  • These results implicate Pax7 as an important determinant of polarity within the mouse superior colliculus, and suggest a role in retinotopic mapping [11].
 

Associations of Pax7 with chemical compounds

  • We have identified alternatively spliced isoforms of murine Pax-3 and Pax-7 which differ by the presence or absence of a single glutamine residue in a linker region which separates two distinct DNA-binding subdomains within the paired domain [12].
 

Regulatory relationships of Pax7

 

Other interactions of Pax7

  • Manipulation of the dominant-negative forms of these factors in satellite cell cultures demonstrates that Pax3 cannot replace the antiapoptotic function of Pax7 [9].
  • 5. In contrast, Pax7 expression is shared among all striated muscles and exhibits a uniform pattern [8].
  • Analysis of the opb mutant phenotype and of opb/opb <--> wild-type chimeric embryos reveals that early in neural development, the wild-type opb gene (opb(+)) is required cell autonomously for the expression of Pax7 in dorsal cells and Pax6 in lateral cells [14].
  • Pax-1 and the human gene HuP48 have identical paired domains, as do Pax-3 and HuP2 as well as Pax-7 and HuP1, and are likely to represent homologous genes in mouse and man [15].
  • Sublaminal cells expressed unambiguous satellite cell markers (M-cadherin, Pax7, NCAM) and fused into scattered GFP+ muscle fibers [16].
 

Analytical, diagnostic and therapeutic context of Pax7

  • In situ hybridization revealed that Pax7 was also expressed in satellite cells residing in adult muscle [4].
  • To investigate the mechanism of these contrasting phenotypes, we replaced Pax3 by Pax7 by using gene targeting in the mouse [17].
  • The expression profiles of Pax7 alternate transcripts were then assessed by RNA isolation and RT-PCR [18].
  • Southern blotting indicated that SJL/J, Quackenbush and DDO mice share a Pax7/TaqI RFLP which differs from all other laboratory strains tested [19].
  • Total RNA was isolated from adult mouse tissues and the polymerase chain reaction was performed on reverse transcribed mRNA using primers specific for regions that encode the paired and homeodomain of Pax7 [20].

References

  1. Pax7 is necessary and sufficient for the myogenic specification of CD45+:Sca1+ stem cells from injured muscle. Seale, P., Ishibashi, J., Scimè, A., Rudnicki, M.A. PLoS Biol. (2004) [Pubmed]
  2. Pax3:Fkhr interferes with embryonic Pax3 and Pax7 function: implications for alveolar rhabdomyosarcoma cell of origin. Keller, C., Hansen, M.S., Coffin, C.M., Capecchi, M.R. Genes Dev. (2004) [Pubmed]
  3. Rhabdomyosarcoma development in mice lacking Trp53 and Fos: tumor suppression by the Fos protooncogene. Fleischmann, A., Jochum, W., Eferl, R., Witowsky, J., Wagner, E.F. Cancer Cell (2003) [Pubmed]
  4. Pax7 is required for the specification of myogenic satellite cells. Seale, P., Sabourin, L.A., Girgis-Gabardo, A., Mansouri, A., Gruss, P., Rudnicki, M.A. Cell (2000) [Pubmed]
  5. Pax3/Pax7 mark a novel population of primitive myogenic cells during development. Kassar-Duchossoy, L., Giacone, E., Gayraud-Morel, B., Jory, A., Gomès, D., Tajbakhsh, S. Genes Dev. (2005) [Pubmed]
  6. Pax3 functions in cell survival and in pax7 regulation. Borycki, A.G., Li, J., Jin, F., Emerson, C.P., Epstein, J.A. Development (1999) [Pubmed]
  7. MyoD-lacZ transgenes are early markers in the neural retina, but MyoD function appears to be inhibited in the developing retinal cells. Kablar, B. Int. J. Dev. Neurosci. (2004) [Pubmed]
  8. Comparative expression analysis of Pax3 and Pax7 during mouse myogenesis. Horst, D., Ustanina, S., Sergi, C., Mikuz, G., Juergens, H., Braun, T., Vorobyov, E. Int. J. Dev. Biol. (2006) [Pubmed]
  9. Pax3 and Pax7 have distinct and overlapping functions in adult muscle progenitor cells. Relaix, F., Montarras, D., Zaffran, S., Gayraud-Morel, B., Rocancourt, D., Tajbakhsh, S., Mansouri, A., Cumano, A., Buckingham, M. J. Cell Biol. (2006) [Pubmed]
  10. Pax3 and Pax7 are expressed in commissural neurons and restrict ventral neuronal identity in the spinal cord. Mansouri, A., Gruss, P. Mech. Dev. (1998) [Pubmed]
  11. Pax7 and superior collicular polarity: insights from Pax6 (Sey) mutant mice. Thompson, J.A., Lovicu, F.J., Ziman, M. Experimental brain research. Experimentelle Hirnforschung. Expérimentation cérébrale (2007) [Pubmed]
  12. An alternative splicing event in the Pax-3 paired domain identifies the linker region as a key determinant of paired domain DNA-binding activity. Vogan, K.J., Underhill, D.A., Gros, P. Mol. Cell. Biol. (1996) [Pubmed]
  13. Myogenic specification of side population cells in skeletal muscle. Asakura, A., Seale, P., Girgis-Gabardo, A., Rudnicki, M.A. J. Cell Biol. (2002) [Pubmed]
  14. Dorsal and lateral fates in the mouse neural tube require the cell-autonomous activity of the open brain gene. Eggenschwiler, J.T., Anderson, K.V. Dev. Biol. (2000) [Pubmed]
  15. Pax: a murine multigene family of paired box-containing genes. Walther, C., Guenet, J.L., Simon, D., Deutsch, U., Jostes, B., Goulding, M.D., Plachov, D., Balling, R., Gruss, P. Genomics (1991) [Pubmed]
  16. Adult bone marrow-derived stem cells in muscle connective tissue and satellite cell niches. Dreyfus, P.A., Chretien, F., Chazaud, B., Kirova, Y., Caramelle, P., Garcia, L., Butler-Browne, G., Gherardi, R.K. Am. J. Pathol. (2004) [Pubmed]
  17. Divergent functions of murine Pax3 and Pax7 in limb muscle development. Relaix, F., Rocancourt, D., Mansouri, A., Buckingham, M. Genes Dev. (2004) [Pubmed]
  18. A key role for Pax7 transcripts in determination of muscle and nerve cells. Ziman, M.R., Thomas, M., Jacobsen, P., Beazley, L. Exp. Cell Res. (2001) [Pubmed]
  19. Association of an unusual form of a Pax7-like gene with increased efficiency of skeletal muscle regeneration. Kay, P.H., Mitchell, C.A., Akkari, A., Papadimitriou, J.M. Gene (1995) [Pubmed]
  20. Alternate Pax7 transcripts are expressed specifically in skeletal muscle, brain and other organs of adult mice. Ziman, M.R., Fletcher, S., Kay, P.H. Int. J. Biochem. Cell Biol. (1997) [Pubmed]
 
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