Laryngeal Muscles Are Spared in the Dystrophin Deficient mdx Mouse 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 ... Research Article
Research Article  |   June 01, 2008
Laryngeal Muscles Are Spared in the Dystrophin Deficient mdx Mouse
 
Author Affiliations & Notes
  • Lisa B. Thomas
    University of Kentucky, Lexington
  • Gayle L. Joseph
    University of Kentucky, Lexington
  • Tracey D. Adkins
    University of Kentucky, Lexington
  • Francisco H. Andrade
    University of Kentucky, Lexington
  • Joseph C. Stemple
    University of Kentucky, Lexington
  • Contact author: Lisa B. Thomas, who is now with the Department of Communication Disorders, Marshall University, 1 John Marshall Drive, Huntington, WV 25755. E-mail: thomasl@marshall.edu.
Article Information
Speech, Voice & Prosody / Speech / Research Articles
Research Article   |   June 01, 2008
Laryngeal Muscles Are Spared in the Dystrophin Deficient mdx Mouse
Journal of Speech, Language, and Hearing Research, June 2008, Vol. 51, 586-595. doi:10.1044/1092-4388(2008/042)
History: Received November 28, 2006 , Accepted August 29, 2007
 
Journal of Speech, Language, and Hearing Research, June 2008, Vol. 51, 586-595. doi:10.1044/1092-4388(2008/042)
History: Received November 28, 2006; Accepted August 29, 2007
Web of Science® Times Cited: 18

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.

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