Excitation–contraction coupling is the physiological mechanism occurring in muscle cells whereby an electrical signal sensed by the dihydropyridine receptor located on the transverse tubules is transformed into a chemical gradient (Ca2+ increase) by activation of the ryanodine receptor located on the sarcoplasmic reticulum membrane. In the present study, we characterized for the first time the excitation–contraction coupling machinery of an immortalized human skeletal muscle cell line. Intracellular Ca2+ measurements showed a normal response to pharmacological activation of the ryanodine receptor, whereas 3D-SIM (super-resolution structured illumination microscopy) revealed a low level of structural organization of ryanodine receptors and dihydropyridine receptors. Interestingly, the expression levels of several transcripts of proteins involved in Ca2+ homoeostasis and differentiation indicate that the cell line has a phenotype closer to that of slow-twitch than fast-twitch muscles. These results point to the potential application of such human muscle-derived cell lines to the study of neuromuscular disorders; in addition, they may serve as a platform for the development of therapeutic strategies aimed at correcting defects in Ca2+ homoeostasis due to mutations in genes involved in Ca2+ regulation.
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Research Article|
September 27 2013
Establishment of a human skeletal muscle-derived cell line: biochemical, cellular and electrophysiological characterization
Ori Rokach;
Ori Rokach
*Department of Anaesthesia, University Hospital Basel, Hebelstrasse 20, 4031 Basel, Switzerland
†Department of Biomedicine, University Hospital Basel, Hebelstrasse 20, 4031 Basel, Switzerland
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Nina D. Ullrich;
Nina D. Ullrich
‡Department of Physiology, University of Bern, Bern, Switzerland
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Martin Rausch;
Martin Rausch
§Novartis Biomedical Institute, Postfach 4002 Basel, Switzerland
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Vincent Mouly;
Vincent Mouly
∥Thérapie des maladies du muscle strié, Institut de Myologie, UM76, UPMC, Université Paris 6, Paris, France
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Haiyan Zhou;
Haiyan Zhou
¶Dubowitz Neuromuscular Centre, Institute of Child Health, University College London, 30 Guilford Street, London WC1N 1EH, U.K.
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Francesco Muntoni;
Francesco Muntoni
¶Dubowitz Neuromuscular Centre, Institute of Child Health, University College London, 30 Guilford Street, London WC1N 1EH, U.K.
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Francesco Zorzato;
Francesco Zorzato
1
*Department of Anaesthesia, University Hospital Basel, Hebelstrasse 20, 4031 Basel, Switzerland
**Department of Life Sciences and Biotechnology, University of Ferrara, Ferrara, Italy
Correspondence may be addressed to either author (email zor@unife.it or susan.treves@unibas.ch).
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Susan Treves
Susan Treves
1
*Department of Anaesthesia, University Hospital Basel, Hebelstrasse 20, 4031 Basel, Switzerland
**Department of Life Sciences and Biotechnology, University of Ferrara, Ferrara, Italy
Correspondence may be addressed to either author (email zor@unife.it or susan.treves@unibas.ch).
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Publisher: Portland Press Ltd
Received:
May 24 2013
Revision Received:
July 10 2013
Accepted:
August 01 2013
Accepted Manuscript online:
August 01 2013
Online ISSN: 1470-8728
Print ISSN: 0264-6021
© The Authors Journal compilation © 2013 Biochemical Society
2013
Biochem J (2013) 455 (2): 169–177.
Article history
Received:
May 24 2013
Revision Received:
July 10 2013
Accepted:
August 01 2013
Accepted Manuscript online:
August 01 2013
Citation
Ori Rokach, Nina D. Ullrich, Martin Rausch, Vincent Mouly, Haiyan Zhou, Francesco Muntoni, Francesco Zorzato, Susan Treves; Establishment of a human skeletal muscle-derived cell line: biochemical, cellular and electrophysiological characterization. Biochem J 15 October 2013; 455 (2): 169–177. doi: https://doi.org/10.1042/BJ20130698
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