Laryngeal muscles are spared in the dystrophin deficient mdx mouse

Lisa B. Thomas, Gayle L. Joseph, Tracey D. Adkins, Francisco H. Andrade, Joseph C. Stemple

Research output: Contribution to journalArticlepeer-review

22 Scopus citations

Abstract

Purpose: Duchenne muscular dystrophy (DMD) is caused by the loss of the cytoskeletal protein, dystrophin. The disease leads to severe and progressive skeletal muscle wasting. Interestingly, the disease spares some muscles. The purpose of the study was to determine the effects of dystrophin deficiency on 2 intrinsic laryngeal muscles, the posterior cricoarytenoid and the thyroarytenoid, in the mouse model. Method: Larynges from dystrophin-deficient mdx and normal mice were examined histologically. Results: Results demonstrate that despite the absence of dystrophin in the mdx laryngeal muscles, membrane damage, inflammation, necrosis, and regeneration were not detected in the assays performed. Conclusions: The authors concluded that these muscles are 1 of only a few muscle groups spared in this model of dystrophin deficiency. The muscles may count on intrinsic and adaptive protective mechanisms to cope with the absence of dystrophin. Identifying these protective mechanisms may improve DMD management. The study also highlights the unique aspects of the selected laryngeal skeletal muscles and their dissimilarity to limb skeletal muscle.

Original languageEnglish
Pages (from-to)586-595
Number of pages10
JournalJournal of Speech, Language, and Hearing Research
Volume51
Issue number3
DOIs
StatePublished - Jun 2008

Keywords

  • Laryngeal
  • Muscles
  • Muscular dystrophy
  • Posterior cricoarytenoid
  • Thyroarytenoid

ASJC Scopus subject areas

  • Language and Linguistics
  • Linguistics and Language
  • Speech and Hearing

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