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 language | English (US) |
|---|---|
| Title of host publication | Handbook of Mouse Auditory Research |
| Subtitle of host publication | From Behavior to Molecular Biology |
| Publisher | CRC Press |
| Pages | 533-535 |
| Number of pages | 3 |
| ISBN (Electronic) | 9781420038736 |
| ISBN (Print) | 0849323282, 9780849323287 |
| State | Published - Jan 1 2001 |
ASJC Scopus subject areas
- Medicine(all)
- Biochemistry, Genetics and Molecular Biology(all)