Modifications in the sarcoplasmic reticulum and subcellular calcium distribution in skeletal muscle in a case of Westphal's disease (hypokalemic periodic paralysis) |
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Authors: | Aimé Porte Marie Elisabeth Stoeckel Jean Yves Ledeaut Sylvie Guez Colette Hindelang-Gertner Gérard Mack |
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Institution: | (1) Laboratoire de Physiologie animale, Département de microscopie électronique, ULP Sciences, Strasbourg, France;(2) Institut de Chimie biologique, ULP Médecine, Strasbourg, France;(3) Pédiatrie II, CHU, Strasbourg, France |
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Abstract: | Summary In a case of hypokalemic periodic paralysis with characteristic alterations of the sarcoplasmic reticulum (SR) in the skeletal muscle, subcellular calcium re-partition, as revealed with the pyroantimonate technique, appears disturbed during paralysis. Pyroantimonate precipitates, normally concentrated in the terminal cisternae of the SR, were localized in the T tubules, whereas the terminal cisternae appeared empty. The increase (about 14%) in muscular calcium during paralysis may result from the accumulation of calcium in the extracellular compartment (T tubules). Defects in calcium uptake and storage by the SR may be. involved in the pathogenesis of the periodic paralysis syndrome. |
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Keywords: | Hypokalemic periodic paralysis Calcium Sarcoplasmic reticulum T tubules |
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