Altered gating and conductance of Na+ channels in hyperkalemic periodic paralysis

F. Lehmann -Horn, P. A. Iaizzo, H. Hatt, Ch Franke

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53 Scopus citations

Abstract

Electrophysiological studies on muscle fibres from patients with hyperkalemic periodic paralysis with myotonia have shown that the episodes of weakness are caused by a sustained depolarization of the sarcolemma to potentials between -40 and -60 mV. In muscle fibre segments from three such patients this sustained depolarization was caused by noninactivating Na+ channels with reduced single-channel conductance blocked by TTX and procainamide. As the chloride conductance was normal, myotonia may be best explained with the abnormal reopenings of the Na+ channels. The recently described genetic linkage between hyperkalemic periodic paralysis with myotonia and the gene coding for the TTX-sensitive Na+ channel suggests an altered primary structure of this channel causing its abnormal function.

Original languageEnglish (US)
Pages (from-to)297-299
Number of pages3
JournalPflügers Archiv European Journal of Physiology
Volume418
Issue number3
DOIs
StatePublished - Apr 1 1991

Keywords

  • Adynamia episodica hereditaria with myotonia
  • Hereditary muscle disease
  • Patch clamp technique
  • Sodium channel disease

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