Dystrophin and utrophin expression require sarcospan: loss of α7 integrin exacerbates a newly discovered muscle phenotype in sarcospan-null mice.

@article{Marshall2012DystrophinAU,
  title={Dystrophin and utrophin expression require sarcospan: loss of α7 integrin exacerbates a newly discovered muscle phenotype in sarcospan-null mice.},
  author={Jamie L. Marshall and Eric Chou and Jennifer Oh and Allan Kwok and Dean J. Burkin and Rachelle H. Crosbie-Watson},
  journal={Human molecular genetics},
  year={2012},
  volume={21 20},
  pages={4378-93}
}
Sarcospan (SSPN) is a core component of the major adhesion complexes in skeletal muscle, the dystrophin- and utrophin (Utr)-glycoprotein complexes (DGC and UGC). We performed a rigorous analysis of SSPN-null mice and discovered that loss of SSPN decreased DGC and UGC abundance, leading to impaired laminin-binding activity and susceptibility to eccentric contraction-induced injury in skeletal muscle. We show that loss of SSPN increased levels of α7β1 integrin. To genetically test whether… CONTINUE READING
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