PMID: 11316257Apr 24, 2001Paper

Voltage-gated sodium channel (SkM1) content in dystrophin-deficient muscle

Pflügers Archiv : European journal of physiology
P RibauxS Blaineau

Abstract

The membrane cytoskeleton is increasingly considered as both an anchor and a functional modulator for ion channels. The cytoskeletal disruptions that occur in the absence of dystrophin led us to investigate the voltage-gated sodium channel (SkM1) content in the extensor digitorum longus (EDL) muscle of the dystrophin-deficient mdx mouse. Levels of SkM1 mRNA were determined by semiquantitative reverse transcriptase polymerase chain reaction (RT-PCR). A C-terminal portion of the mouse-specific SkM1 alpha-subunit cDNA (mScn4a) was identified first. SkM1 mRNA levels were as abundant in mdx as in normal muscle, thus suggesting that the transcriptional rate of SkM1 remains unchanged in mdx muscle. However, SkMI density in the extrajunctional sarcolemma was shown to be significantly reduced in mdx muscle, using confocal immunofluorescence image analysis. This decrease was found to be associated with a reduction in the number of SkM1-rich fast-twitch IIb fibres in mdx muscle. In addition, lowered SkM1 sarcolemmal labelling was found in all mdx fibres regardless of their metabolic type. These results suggest the existence of a perturbation of SkM1 anchorage to the plasma membrane. Such an alteration is likely to be related to the 50% de...Continue Reading

Citations

Aug 16, 2008·Pflügers Archiv : European journal of physiology·M MilleK Hilber
Jun 14, 2002·Neuromuscular Disorders : NMD·Alan E H Emery
Jul 16, 2008·The Journal of General Physiology·Carole HirnUrs T Ruegg
Feb 5, 2010·American Journal of Physiology. Cell Physiology·Joana CapoteJulio L Vergara
Jul 1, 2006·Future Cardiology·Matteo Vatta, Georgine Faulkner
Jun 13, 2006·Journal of Cardiovascular Electrophysiology·Jeffrey A Towbin, Neil E Bowles
Jan 23, 2008·Scandinavian Journal of Medicine & Science in Sports·H PiitulainenH Kyröläinen
Mar 30, 2010·Heart Failure Clinics·Roy BeinartDavid Milan
Apr 3, 2016·The European Journal of Neuroscience·Elizabeth M van der PijlJaap J Plomp
Jul 29, 2006·Journal of Muscle Research and Cell Motility·Bruno Allard
Oct 26, 2010·Journal of Muscle Research and Cell Motility·Ian Curtis SmithRene Vandenboom
Sep 27, 2005·Physiological Reviews·Jeanne M Nerbonne, Robert S Kass
Nov 14, 2002·The Journal of Biological Chemistry·Yijun OuGianrico Farrugia

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