Decreased osmotic stability of dystrophin-less muscle cells from the mdx mouse (original) (raw)

Nature volume 349, pages 69–71 (1991)Cite this article

Abstract

HUMAN X-linked Duchenne and Becker muscular dystrophies are due to defects in dystrophin, the product of an exceptionally large gene1,2. Although dystrophin has been characterized as a spectrin-like3 submembranous4 cytoskeletal protein, there is no experimental evidence for its function in the structural maintenance of muscle5. Current hypotheses attribute necrosis of dystrophin-less fibres in situ to mechanical weakening of the outer membrane6, to an excessive influx of Ca2+ions7,8, or to a combination of these two mechanisms, possibly mediated by stretch-sensitive ion channels9. Using hypo-osmotic shock to determine stress resistance10 and a mouse model (mdx)11,12 for the human disease, we show that functional dystrophin contributes to the stability of both cultured myotubes and isolated mature muscle fibres.

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  1. Developmental Biology Unit, University of Bielefeld, POB 8640, W-4800, Bielefeld, 1, Germany
    A. Menke & H. Jockusch

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  1. A. Menke
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  2. H. Jockusch
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Menke, A., Jockusch, H. Decreased osmotic stability of dystrophin-less muscle cells from the mdx mouse.Nature 349, 69–71 (1991). https://doi.org/10.1038/349069a0

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