Focus: Muscular dystrophy mouse models and hearing deficits

De Ann M. Pillers, Dennis R. Trune

Research output: Chapter in Book/Report/Conference proceedingChapter

1 Scopus citations


Hearing loss has been demonstrated in association with various forms of muscular dystrophy. A unifying hypothesis regarding the relative roles of structural proteins in the etiology of the various muscular dystrophies has been emerging which may have an impact on the understanding of mechanisms involved in sensorineural deafness. Dystrophin, the product of the DMD gene, is thought to play a structural role in cells by linking the actin cytoskeleton to a transmembrane cluster of proteins known as the “dystrophin glycoprotein complex” or DGC. The DGC, in turn, is linked to the basement membrane via laminin (Ervasti and Campbell, 1991). Proteins at each level of the association have been linked to muscular dystrophy phenotypes (Bonneman et al., 1996) and may also be necessary for cochlear function.

Original languageEnglish (US)
Title of host publicationHandbook of Mouse Auditory Research
Subtitle of host publicationFrom Behavior to Molecular Biology
PublisherCRC Press
Number of pages3
ISBN (Electronic)9781420038736
ISBN (Print)0849323282, 9780849323287
StatePublished - Jan 1 2001

ASJC Scopus subject areas

  • Medicine(all)
  • Biochemistry, Genetics and Molecular Biology(all)


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