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EFFECT OF DYSTROPHIN DEFICIENCY ON SELECTED INTRINSIC LARYNGEAL MUSCLES OF THE mdx MOUSE

The intrinsic laryngeal muscles are recognized as a highly specialized allotype of skeletal muscle. To date, much of the research examining the properties of this muscle group has been conducted on 2 primary muscles: the thyroarytenoid and posterior cricoarytenoid. Consequently, it is unknown whether the remaining intrinsic laryngeal muscles evidence this highly refined phenotype or if they retain a phenotype more similar to prototypical skeletal muscle. The purpose of this study was to further define the biologic properties of the interarytenoid (IA) and cricothyroid (CT) muscles of the larynx using the dystrophin deficient mdx mouse model. Previous work in this model has demonstrated sparing of select craniofacial muscles in the disease. Interestingly, a vast body of literature also supports the uniqueness of these spared muscles in a number of other areas including: fiber types, motor unit size, proprioceptive mechanisms, myosin isoform expression, remodeling behaviors, and sarcomeric structure. It follows, then, that muscle response to dystrophin deficiency serves as a sensitive marker of a muscles level of biological specialization and its similarity to or departure from classic limb muscle. Larynges and gastrocnemius muscles from 8 mdx and 8 C57BL control mice were examined histologically for typical markers of dystrophinopathy. Immunocytochemical testing examined the distribution of dystrophin and its homolog, utrophin, in control and mdx muscles. Results demonstrated that despite the absence of dystrophin, the laryngeal muscles did not show the classic markers of disease. The mdx superior cricoarytenoid muscle (SCA; mouse counterpart of human IA) demonstrated no evidence of damage, inflammation, necrosis, or regeneration. The mdx CT evidenced subtle markers of regeneration (eg, slight increase in centrally nucleated fibers) but no evidence of degeneration. The authors concluded that the SCA was spared from the effects of dystrophin deficiency, while the CT was strongly protected. The results demonstrate that the SCA and CT muscles of the larynx possess a specialized nature that separates them from prototypical limb muscle. Information from the study offers insight into the unique biology of the laryngeal muscles and holds implications for the translational study of voice and voice disorders.

Identiferoai:union.ndltd.org:uky.edu/oai:uknowledge.uky.edu:gradschool_diss-1594
Date01 January 2008
CreatorsThomas, Lisa Beth
PublisherUKnowledge
Source SetsUniversity of Kentucky
Detected LanguageEnglish
Typetext
Formatapplication/pdf
SourceUniversity of Kentucky Doctoral Dissertations

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