Sarcospan reduces dystrophic pathology: stabilization of the utrophin–glycoprotein complex

@article{Peter2008SarcospanRD,
  title={Sarcospan reduces dystrophic pathology: stabilization of the utrophin–glycoprotein complex},
  author={Angela K. Peter and Jamie L. Marshall and Rachelle H. Crosbie},
  journal={The Journal of Cell Biology},
  year={2008},
  volume={183},
  pages={419 - 427}
}
Mutations in the dystrophin gene cause Duchenne muscular dystrophy and result in the loss of dystrophin and the entire dystrophin-glycoprotein complex (DGC) from the sarcolemma. We show that sarcospan (SSPN), a unique tetraspanin-like component of the DGC, ameliorates muscular dystrophy in dystrophin-deficient mdx mice. SSPN stabilizes the sarcolemma by increasing levels of the utrophin-glycoprotein complex (UGC) at the extrasynaptic membrane to compensate for the loss of dystrophin. Utrophin… CONTINUE READING

Citations

Publications citing this paper.
Showing 1-10 of 24 extracted citations

References

Publications referenced by this paper.
Showing 1-10 of 18 references

Assembly of the dystrophin - associated protein complex does not require the dystrophin COOH - terminal domain

  • J. A. Faulkner, R. H. Crosbie, K. P. Campbell, S. C. Froehner, J. S. Chamberlain
  • Nature .
  • 2000

Similar Papers

Loading similar papers…