The survival motor neurons protein uses its ZPR for nuclear localization (original) (raw)

Nature Cell Biology volume 3, pages E93–E94 (2001)Cite this article

Spinal muscular atrophy is caused by mutations in the survival motor neurons gene, SMN1. The SMN protein localizes to both the cytoplasm and the nuclear bodies. One hallmark of spinal muscular atrophy is the failure of SMN to localize within these nuclear bodies. A recent study has identified a protein called ZPR1 that is required for nuclear translocation of SMN.

This is a preview of subscription content, access via your institution

Access options

Subscribe to this journal

Receive 12 print issues and online access

$209.00 per year

only $17.42 per issue

Buy this article

Prices may be subject to local taxes which are calculated during checkout

Additional access options:

Figure 1: Model of ZPR1 interaction with SMN.

References

  1. Wirth, B. Hum. Mutat. 15, 228–237 (2000).
    Article CAS Google Scholar
  2. Lefebvre, S. et al. Cell 80, 155–165 (1995).
    Article CAS Google Scholar
  3. Lorson, C. L., Hahnen, E., Androphy, E. J. & Wirth, B. Proc. Natl Acad. Sci. USA 96, 6307– 6311 (1999).
    Article CAS Google Scholar
  4. Frugier, T. et al. Hum. Mol. Genet. 9, 849– 858 (2000).
    Article CAS Google Scholar
  5. Gangwani, L., Mikrut, M., Galcheva-Gargova, Z. & Davis, R. J. J Cell Biol 143, 1471–1484 (1998).
    Article CAS Google Scholar
  6. Galcheva-Gargova, Z. et al. Science 272, 1797– 1802 (1996).
    Article CAS Google Scholar
  7. Schlessinger, J. Cell 103, 211–225 ( 2000).
    Article CAS Google Scholar
  8. Galcheva-Gargova, Z. et al. Mol. Biol. Cell 9, 2963– 2971 (1998).
    Article CAS Google Scholar
  9. Gangwani, L., Mikrut, M., Theroux, S., Sharma, M. & Davis, R. J. Nature Cell Biol. 3, 376– 383 (2001).
    Article CAS Google Scholar
  10. Matera, A. G. Trends Cell Biol. 9, 302–309 (1999).
    Article CAS Google Scholar
  11. Fischer, U., Liu, Q. & Dreyfuss, G. Cell 90, 1023– 1029 (1997).
    Article CAS Google Scholar
  12. Pellizzoni, L., Charroux, B., Rappsilber, J., Mann, M. & Dreyfuss, G. J. Cell. Biol. 152, 75–86 (2001).
    Article CAS Google Scholar
  13. Friesen, W. J. & Dreyfuss, G. J. Biol. Chem. 275, 26370–26375 ( 2000).
    Article CAS Google Scholar
  14. Charroux, B. et al. J. Cell Biol. 148, 1177– 1186 (2000).
    Article CAS Google Scholar
  15. Gall, J. G. Annu. Rev. Cell Dev. Biol. 16, 273– 300 (2000).
    Article CAS Google Scholar
  16. Francis, J. W., Sandrock, A. W., Bhide, P. G., Vonsattel, J. P. & Brown, R. H. J. Proc. Natl Acad. Sci. USA 95, 6492–6497 (1998).
    Article CAS Google Scholar

Download references

Author information

Authors and Affiliations

  1. Department of Genetics, Center for Human Genetics and Program in Cell Biology Case Western Reserve University and University Hospitals of Cleveland, Cleveland, 44106-4955, OH, USA
    A. Gregory Matera & Michael D. Hebert

Authors

  1. A. Gregory Matera
    You can also search for this author inPubMed Google Scholar
  2. Michael D. Hebert
    You can also search for this author inPubMed Google Scholar

Rights and permissions

About this article

Cite this article

Matera, A., Hebert, M. The survival motor neurons protein uses its ZPR for nuclear localization .Nat Cell Biol 3, E93–E94 (2001). https://doi.org/10.1038/35070157

Download citation