Possible role of apamin-sensitive K+ channels in myotonic dystrophy

Muscle Nerve. 1994 Nov;17(11):1264-70. doi: 10.1002/mus.880171104.

Abstract

Myotonic muscular dystrophy is a genetic disease characterized mainly by muscle atrophy and myotonia, a repetitive electrical activity of muscle. In the present study, the possible role of apamin-sensitive K+ channels in the genesis of myotonia was investigated. Apamin is a peptide from bee venom that specifically blocks small conductance Ca(2+)-activated K+ channels. The injection of a small amount of apamin (20-30 microliters, 10 mumol/L) into the thenar muscle of myotonic dystrophy patients decreased the basal electrical activity during the electromyogram in the 6 patients studied. Myotonic discharges after muscle percussion were more difficult to trigger and of smaller intensity and duration. In 2 controls and in 2 patients with generalized myotonia, as well as in 1 patient with myotonia congenita (where the defect is in chloride channels), apamin had no effect. These results suggest that apamin-sensitive K+ channels participate in the mechanism that generates myotonia in myotonic dystrophy.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Apamin / pharmacology*
  • Electric Conductivity
  • Electromyography
  • Electrophysiology
  • Female
  • Humans
  • Male
  • Middle Aged
  • Myotonic Dystrophy / metabolism*
  • Potassium Channels / drug effects
  • Potassium Channels / physiology*

Substances

  • Potassium Channels
  • Apamin