Decreased osmotic stability of dystrophin-less muscle cells from the mdx mouse - PubMed (original) (raw)
. 1991 Jan 3;349(6304):69-71.
doi: 10.1038/349069a0.
Affiliations
- PMID: 1985268
- DOI: 10.1038/349069a0
Decreased osmotic stability of dystrophin-less muscle cells from the mdx mouse
A Menke et al. Nature. 1991.
Abstract
Human X-linked Duchenne and Becker muscular dystrophies are due to defects in dystrophin, the product of an exceptionally large gene. Although dystrophin has been characterized as a spectrin-like submembranous cytoskeletal protein, there is no experimental evidence for its function in the structural maintenance of muscle. Current hypotheses attribute necrosis of dystrophin-less fibres in situ to mechanical weakening of the outer membrane, to an excessive influx of Ca2+ ions, or to a combination of these two mechanism, possibly mediated by stretch-sensitive ion channels. Using hypo-osmotic shock to determine stress resistance and a mouse model (mdx) for the human disease, we show that functional dystrophin contributes to the stability of both cultured myotubes and isolated mature muscle fibres.
Comment in
- Muscle damage in mdx mice.
Jackson MJ, McArdle A, Edwards RH, Jones DA. Jackson MJ, et al. Nature. 1991 Apr 25;350(6320):664. doi: 10.1038/350664b0. Nature. 1991. PMID: 2023630 No abstract available.
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