edoc

Establishment of a human skeletal muscle-derived cell line : biochemical, cellular and electrophysiological characterization

Rokach, O. and Ullrich, N. D. and Rausch, M. and Mouly, V. and Zhou, H. and Muntoni, F. and Zorzato, F. and Treves, S.. (2013) Establishment of a human skeletal muscle-derived cell line : biochemical, cellular and electrophysiological characterization. Biochemical Journal , 455 (2). pp. 169-177.

[img]
Preview
PDF - Accepted Version
3965Kb

Official URL: http://edoc.unibas.ch/dok/A6338031

Downloads: Statistics Overview

Abstract

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.
Faculties and Departments:03 Faculty of Medicine > Departement Biomedizin > Department of Biomedicine, University Hospital Basel > Perioperative Patient Safety (Girard/Treves)
UniBasel Contributors:Treves, Susan
Item Type:Article, refereed
Article Subtype:Research Article
Publisher:Portland Press
ISSN:0264-6021
e-ISSN:1470-8728
Note:Publication type according to Uni Basel Research Database: Journal article
Language:English
Identification Number:
Last Modified:31 May 2017 09:46
Deposited On:06 Mar 2015 07:44

Repository Staff Only: item control page