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| DECREASED OSMOTIC STABILITY OF DYSTROPHIN-LESS MUSCLE-CELLS FROM THE MDX MOUSE |
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| Author(s): MENKE A, JOCKUSCH H |
| Source: NATURE Volume: 349 Issue: 6304 Pages: 69-71 Published: JAN 3 1991 |
| Times Cited: 243 References: 22 |
| 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 hystrophin-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. |
| Document Type: Article |
| Language: English |
| Reprint Address: MENKE, A (reprint author), UNIV BIELEFELD, DEV BIOL UNIT, W-4800 BIELEFELD 1, GERMANY |
| Publisher: MACMILLAN MAGAZINES LTD, 4 LITTLE ESSEX STREET, LONDON, ENGLAND WC2R 3LF |
| Subject Category: Multidisciplinary Sciences |
| IDS Number: EQ601 |
| ISSN: 0028-0836 |
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| | | | | | | | | Record from Web of Science® | | | | | | | | | |