Abstract
Dystrophin is a cytoskeletal protein associated with the sarcolemma. Genetic depletion of the protein leads to Duchenne muscular dystrophy (DMD) and dystrophic conditions in animals such as the mdx mouse9. Mechanical weakness of the sarcolemma during the stress of contraction can account for an abnormal calcium entry in the cell that in turn triggers proteolysis-dependent dystrophic muscle necrosis5,7,9. This mechanism can also account, in a positive feedback loop, for the increased activity of calcium-selective leak ion channels recorded in dystrophinless muscle fibers15. The increased cytosolic calcium is also responsible for longer calcium transients in the dystrophinless fibers7,14, which may affect muscle contractile properties. In skeletal muscle, contraction is triggered mainly by the calcium ions released from the sarcoplasmic reticulum due to depolarization of the sarcolemma and transverse tubules11. Nevertheless, higher cytosolic calcium levels can affect the rate of calcium release and reuptake1,14, thus modifying the excitation-contraction coupling mechanism.
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References
De Luca, A., Piemo, S., and Conte Camerino, D., 1996, Effect of taurine depletion on excitation contraction coupling and Cl conductance of rat skeletal muscle, Eur. J. Pharmacol, 296:215–222.
De Luca, A., Piemo, S., and Conte Camerino, D., 1997, Electrical properties of diaphragm and EDL muscles during the life of dystrophic mice, Am. J. Physiol. 272 (Cell Physiol. 41): C333–C340.
Dulhunty, A., 1988, Internal citrate ions reduce the membrane potential for contraction threshold in mammalian skeletal muscle fibers, Biophys. J., 53:609–615.
Engel, A.G., Yamamoto, M., and Fischbeck, K.H., 1994, Dystrophinopathies, in: Myology, Engel, A.G. and Franzini-Armstrong, C. eds., Mc Graw-Hill, Inc., New York, Vol. 2, pp. 1133–1187.
Franco-Obregon, A., and Lansman, J.B., 1994, Mechanosensitive ion channels in skeletal muscle from normal and dystrophic mice, J. Physiol., 481,2: 299–309.
Huxtable, R.J., 1992, The physiological actions of taurine, Physiol. Rev. 72, 101–163.
Imbert, N., Cognard, C., Duport, G., Guillon, C., and Raymond, G., Abnormal calcium homeostasis in Duchenne muscular dystrophy myotubes contracting in vitro, Cell Calcium, 18: 177-186.
Lake, N., 1992, Effects of taurine deficiency on arrhythmogenesis and excitation-contraction coupling in cardiac tissues, in Adv. Exp. Med. Biol. Taurine, Nutritional Value and Mechanism of Action, Lombardini J.B., Schaffer S.W. and Azuma J. eds., Plenum Press, New York, Vol. 315, pp. 173–176.
Matsumura, K., and Campbell, K.P., 1994, Dystrophin-glycoprotein complex: its role in the molecular pathogenesis of muscular dystrophies, Muscle Nerve, 17:2–15.
Menke, A. and Jockusch, H., 1991, Decreased osmotic stability of dystrophin-less muscle cells from the mdx mouse, Nature, 349:69–71.
Rios, E., and Pizarro, G., 1991, Voltage sensor of excitation-contraction coupling in skeletal muscle, Physiol. Rev., 71:849–860.
Schaffer, S.W and Azuma, J., 1992, Review: myocardial physiological effects of taurine and their significance, in Adv. Exp. Med. Biol. Taurine, Nutritional Value and Mechanism of Action, Lombardini J.B., Schaffer S.W. and Azuma J. eds., Plenum Press, New York, Vol. 315, pp. 105–120.
Takagi, A., Kojima, S., Ida M., and Araki, M., 1992, Increased leakage of calcium ion from the sarcoplasmic reticulum of the mdx mouse, J. Neurol. Sci., 110:160–164.
Turner, P.R., Fong, P., Denetclaw, W.F., and Steinhardt, R.A., 1991, Increased calcium influx in dystrophic muscle, J. Cell Biol., 115:1701–1712.
Turner, P.R., Schultz, R., Ganguly, B., and Steinhardt, R.A., 1993, Proteolysis results in altered leak channel kinetics and elevated free calcium in mdx muscle, J. Membr. Biol. 133:243–251.
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De Luca, A., Pierno, S., Camerino, C., Huxtable, R.J., Camerino, D.C. (1998). Effect of Taurine on Excitation-Contraction Coupling of Extensor Digitorum Longus Muscle of Dystrophic MDX Mouse. In: Schaffer, S., Lombardini, J.B., Huxtable, R.J. (eds) Taurine 3. Advances in Experimental Medicine and Biology, vol 442. Springer, Boston, MA. https://doi.org/10.1007/978-1-4899-0117-0_15
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DOI: https://doi.org/10.1007/978-1-4899-0117-0_15
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