Constitutive properties, not molecular adaptations, mediate extraocular muscle sparing in dystrophic mdx mice.

@article{Porter2003ConstitutivePN,
  title={Constitutive properties, not molecular adaptations, mediate extraocular muscle sparing in dystrophic mdx mice.},
  author={John David Henley Porter and Anita P. Merriam and Sangeeta Khanna and Francisco Andrade and Chelliah R. Richmonds and P. Michael Leahy and Georgiana Cheng and Paraskevi Karathanasis and Xiaohua Zhou and Linda L Kusner and Marvin E. Adams and Michael Willem and Ulrike Mayer and Henry J. Kaminski},
  journal={FASEB journal : official publication of the Federation of American Societies for Experimental Biology},
  year={2003},
  volume={17 8},
  pages={
          893-5
        }
}
Extraocular muscle (EOM) is spared in Duchenne muscular dystrophy. Here, we tested putative EOM sparing mechanisms predicted from existing dystrophinopathy models. Data show that mdx mouse EOM contains dystrophin-glycoprotein complex (DGC)-competent and DGC-deficient myofibers distributed in a fiber type-specific pattern. Up-regulation of a dystrophin homologue, utrophin, mediates selective DGC retention. Counter to the DGC mechanical hypothesis, an intact DGC is not a precondition for EOM… CONTINUE READING
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