Focus

Muscular dystrophy mouse models and hearing deficits

De Ann M. Pillers, Dennis Trune

Research output: Chapter in Book/Report/Conference proceedingChapter

1 Citation (Scopus)

Abstract

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
Pages533-535
Number of pages3
ISBN (Electronic)9781420038736
ISBN (Print)0849323282, 9780849323287
StatePublished - Jan 1 2001

Fingerprint

Muscular Dystrophies
Audition
Hearing
Dystrophin
Proteins
Cochlea
Deafness
Laminin
Actin Cytoskeleton
Hearing Loss
Basement Membrane
Actins
Glycoproteins
Genes
Phenotype

ASJC Scopus subject areas

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

Cite this

Pillers, D. A. M., & Trune, D. (2001). Focus: Muscular dystrophy mouse models and hearing deficits. In Handbook of Mouse Auditory Research: From Behavior to Molecular Biology (pp. 533-535). CRC Press.

Focus : Muscular dystrophy mouse models and hearing deficits. / Pillers, De Ann M.; Trune, Dennis.

Handbook of Mouse Auditory Research: From Behavior to Molecular Biology. CRC Press, 2001. p. 533-535.

Research output: Chapter in Book/Report/Conference proceedingChapter

Pillers, DAM & Trune, D 2001, Focus: Muscular dystrophy mouse models and hearing deficits. in Handbook of Mouse Auditory Research: From Behavior to Molecular Biology. CRC Press, pp. 533-535.
Pillers DAM, Trune D. Focus: Muscular dystrophy mouse models and hearing deficits. In Handbook of Mouse Auditory Research: From Behavior to Molecular Biology. CRC Press. 2001. p. 533-535
Pillers, De Ann M. ; Trune, Dennis. / Focus : Muscular dystrophy mouse models and hearing deficits. Handbook of Mouse Auditory Research: From Behavior to Molecular Biology. CRC Press, 2001. pp. 533-535
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