Decreased osmotic stability of dystrophin-less muscle cells from the mdx mouse

Menke A, Jockusch H (1991)
Nature 349(6304): 69-71.

Journal Article | Published | English

No fulltext has been uploaded

Author
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.
Publishing Year
ISSN
PUB-ID

Cite this

Menke A, Jockusch H. Decreased osmotic stability of dystrophin-less muscle cells from the mdx mouse. Nature. 1991;349(6304):69-71.
Menke, A., & Jockusch, H. (1991). Decreased osmotic stability of dystrophin-less muscle cells from the mdx mouse. Nature, 349(6304), 69-71.
Menke, A., and Jockusch, H. (1991). Decreased osmotic stability of dystrophin-less muscle cells from the mdx mouse. Nature 349, 69-71.
Menke, A., & Jockusch, H., 1991. Decreased osmotic stability of dystrophin-less muscle cells from the mdx mouse. Nature, 349(6304), p 69-71.
A. Menke and H. Jockusch, “Decreased osmotic stability of dystrophin-less muscle cells from the mdx mouse”, Nature, vol. 349, 1991, pp. 69-71.
Menke, A., Jockusch, H.: Decreased osmotic stability of dystrophin-less muscle cells from the mdx mouse. Nature. 349, 69-71 (1991).
Menke, A, and Jockusch, Harald. “Decreased osmotic stability of dystrophin-less muscle cells from the mdx mouse”. Nature 349.6304 (1991): 69-71.
This data publication is cited in the following publications:
This publication cites the following data publications:

135 Citations in Europe PMC

Data provided by Europe PubMed Central.

Membrane-stabilizing copolymers confer marked protection to dystrophic skeletal muscle in vivo.
Houang EM, Haman KJ, Filareto A, Perlingeiro RC, Bates FS, Lowe DA, Metzger JM., Mol Ther Methods Clin Dev 2(), 2015
PMID: 26623440
Pregnancy-induced amelioration of muscular dystrophy phenotype in mdx mice via muscle membrane stabilization effect of glucocorticoid.
Shimizu-Motohashi Y, Asakura Y, Motohashi N, Belur NR, Baumrucker MG, Asakura A., PLoS ONE 10(3), 2015
PMID: 25775477
Elusive sources of variability of dystrophin rescue by exon skipping.
Vila MC, Klimek MB, Novak JS, Rayavarapu S, Uaesoontrachoon K, Boehler JF, Fiorillo AA, Hogarth MW, Zhang A, Shaughnessy C, Gordish-Dressman H, Burki U, Straub V, Lu QL, Partridge TA, Brown KJ, Hathout Y, van den Anker J, Hoffman EP, Nagaraju K., Skelet Muscle 5(), 2015
PMID: 26634117
α1-Syntrophin-deficient mice exhibit impaired muscle force recovery after osmotic shock.
Yokota T, Miyagoe-Suzuki Y, Ikemoto T, Matsuda R, Takeda S., Muscle Nerve 49(5), 2014
PMID: 24037898
Atomic force microscopy of 3T3 and SW-13 cell lines: an investigation of cell elasticity changes due to fixation.
Codan B, Martinelli V, Mestroni L, Sbaizero O., Mater Sci Eng C Mater Biol Appl 33(6), 2013
PMID: 23706214
Constructing reference ranges.
Howel D., Muscle Nerve 16(12), 1993
PMID: 8232400
Duchenne muscular dystrophy.
Chamberlain JS., Curr. Opin. Genet. Dev. 1(1), 1991
PMID: 1840869

Export

0 Marked Publications

Open Data PUB

Web of Science

View record in Web of Science®

Sources

PMID: 1985268
PubMed | Europe PMC

Search this title in

Google Scholar