Enhanced excitation-coupled Ca(2+) entry induces nuclear translocation of NFAT and contributes to IL-6 release from myotubes from patients with central core disease.

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Serval ID
serval:BIB_AF80EAD1E0C4
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Enhanced excitation-coupled Ca(2+) entry induces nuclear translocation of NFAT and contributes to IL-6 release from myotubes from patients with central core disease.
Journal
Human Molecular Genetics
Author(s)
Treves S., Vukcevic M., Jeannet P.Y., Levano S., Girard T., Urwyler A., Fischer D., Voit T., Jungbluth H., Lillis S., Muntoni F., Quinlivan R., Sarkozy A., Bushby K., Zorzato F.
ISSN
1460-2083[electronic], 0964-6906[linking]
Publication state
Published
Issued date
2011
Volume
20
Number
3
Pages
589-600
Language
english
Abstract
Prolonged depolarization of skeletal muscle cells induces entry of extracellular calcium into muscle cells, an event referred to as excitation-coupled calcium entry. Skeletal muscle excitation-coupled calcium entry relies on the interaction between the 1,4-dihydropyridine receptor on the sarcolemma and the ryanodine receptor on the sarcoplasmic reticulum membrane. In this study, we directly measured excitation-coupled calcium entry by total internal reflection fluorescence microscopy in human skeletal muscle myotubes harbouring mutations in the RYR1 gene linked to malignant hyperthermia (MH) and central core disease (CCD). We found that excitation-coupled calcium entry is strongly enhanced in cells from patients with CCD compared with individuals with MH and controls. Furthermore, excitation-coupled calcium entry induces generation of reactive nitrogen species and enhances nuclear localization of NFATc1, which in turn may be responsible for the increased IL-6 released by myotubes from patients with CCD.
Pubmed
Web of science
Open Access
Yes
Create date
15/02/2011 13:28
Last modification date
14/02/2022 7:56
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