Localization of Muscle Gene Products in Nuclear Domains: Does this Constitute a Problem for Myoblast Therapy?

  • Helen M. Blau
  • Grace K. Pavlath
  • Kevin Rich
  • Steven G. Webster
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 280)


A question of major interest in considering myoblast therapy is whether the gene product dystrophin, provided by the introduced myoblasts, can contribute to the function of the myofiber into which the cells fuse. As shown in several papers in this volume, there is now ample evidence that injected myoblasts can fuse with myofibers and produce dystrophin, but the question of whether that dystrophin can restore muscle function still remains. In this regard, a major consideration is whether dystrophin can gain access to distant sites within the myofiber or remains localized in the vicinity of the nucleus that encoded it.


Duchenne Muscular Dystrophy Muscle Gene Elevated Creatine Kinase Nuclear Domain Myoblast Transplantation 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 1.
    Ralston, E. and Z.W. Hall, Transfer of a protein encoded by a single nucleus to nearby nuclei in multinucleated myotubes, Science, 244: 1066 (1989).PubMedCrossRefGoogle Scholar
  2. 2.
    Blau, H.M., C.-P. Chiu and C. Webster, Cytoplasmic activation of human nuclear genes in stable heterocaryons, Cell 32: 117 (1983).CrossRefGoogle Scholar
  3. 3.
    Blau, H.M., G.K. Pavlath, E.C. Hardeman, C.-P. Chiu, L. Silberstein, S.G. Webster, S.C. Miller and C. Webster, Plasticity of the differentiated state, Science 230: 758 (1985).PubMedCrossRefGoogle Scholar
  4. 4.
    Walsh, F.S., G. Dickson, S.E. Moore, C.H. Barton, Unmasking N-CAM, Nature 339: 516 (1989).PubMedCrossRefGoogle Scholar
  5. 5.
    Pavlath, G.K., K. Rich, S.G. Webster and H.M. Blau, Localization of muscle gene products in nuclear domains, Nature 337: 570 (1989).PubMedCrossRefGoogle Scholar
  6. 6.
    Watkins, J.F. and D.M. Grace, Studies on the surface antigens of interspecific mammalian cell heterokaryons, J. Cell Sci. 2: 193 (1967).PubMedGoogle Scholar
  7. 7.
    Harris, H., E. Sidebottom, D.M. Grace and M.E. Bramwell, The expression of genetic information: A study with hybrid animal cells, J. Cell Sci. 4: 499 (1969).PubMedGoogle Scholar
  8. 8.
    Frye, L.D. and M. Edidin, The rapid intermixing of cell surface antigens after formation of mouse-human heterokaryons, J. Cell Sci., 7: 319 (1970).PubMedGoogle Scholar
  9. 9.
    Tassin, A.-M., B. Mara and M. Bornens, Fate of microtubule - organizing centers during myogenesis in vitro,J. Cell Biol. 100: 35 (1985).PubMedCrossRefGoogle Scholar
  10. 10.
    Partridge, T.A., M. Grounds and J.C. Sloper, Evidence of fusion between host and donor myoblasts in skeletal muscle grafts, Nature 273: 306 (1978).PubMedCrossRefGoogle Scholar
  11. 11.
    Gearhart, J.D. and B. Mintz, Clonal origins of somites and their muscle derivatives; evidence from allophenic mice,Develop. Biol. 29: 27 (1972).Google Scholar
  12. 12.
    Hausmanowa- Petrusewicz, I., I. Niebroj- Dobosz, J. Borkowska, E. Lukasik and D. Liszewska-Pfejfer, Carrier detection in Duchenne dystrophy in: “Pathogenesis of Human Muscular Dystrophies,” L.P. Rowland, ed., Excerpta Medica, Oxford (1977).Google Scholar
  13. 13.
    Koenig, M., A.P. Monaco and L.M. Kunkel, The complete sequence of dystrophin predicts a rod-shaped cytoskeletal protein, Cell 53: 219 (1988).PubMedCrossRefGoogle Scholar
  14. 14.
    Miller, S.C., G.K. Pavlath, B.T. Blakely and H.M. Blau, Muscle cell components dictate hepatocyte gene expression and the distribution of the Golgi apparatus in heterokaryons, Genes Devel., 2: 330 (1988).PubMedCrossRefGoogle Scholar
  15. 15.
    Silberstein, L., S.G. Webster, M. Travis and H.M. Blau, Developmental progression of myosin gene expression in cultured muscle cells, Cell, 46: 1075 (1986).PubMedCrossRefGoogle Scholar
  16. 16.
    Webster, C., L. Silberstein, A.P. Hays and H.M. Blau, Fast muscle fibers are preferentially affected in Duchenne muscular dystrophy, Cell, 52: 502 (1988).Google Scholar

Copyright information

© Plenum Press, New York 1990

Authors and Affiliations

  • Helen M. Blau
    • 1
  • Grace K. Pavlath
    • 1
  • Kevin Rich
    • 1
  • Steven G. Webster
    • 1
  1. 1.Department of PharmacologyStanford University School of MedicineStanfordUSA

Personalised recommendations