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Abnormal ryanodine receptor channels in malignant hyperthermia.

M Fill1, R Coronado, J R Mickelson

  • 1Department of Physiology and Molecular Biophysics, Baylor College of Medicine, Houston, Texas 77030.

Biophysical Journal
|March 1, 1990
PubMed
Summary

Malignant hyperthermia (MH) susceptibility is linked to altered calcium release from the sarcoplasmic reticulum (SR). MHS channels show abnormal calcium inactivation, suggesting a defect in a low-affinity calcium binding site.

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Area of Science:

  • Muscle Physiology
  • Molecular Biology
  • Genetics

Background:

  • Previous research indicates a defect in sarcoplasmic reticulum (SR) calcium release in individuals susceptible to malignant hyperthermia (MH).
  • The precise nature of this defect, particularly concerning the SR calcium release channel, remains to be fully elucidated.

Purpose of the Study:

  • To investigate alterations in SR calcium release channels in porcine muscle susceptible to MH.
  • To compare the calcium (Ca2+) dependence of channel gating in normal versus MH-susceptible (MHS) muscle.

Main Methods:

  • Purification of SR vesicles from normal and MHS porcine muscle.
  • Measurement of 45Ca2+ efflux rates from SR vesicles.
  • Single-channel recordings of SR vesicles incorporated into planar lipid bilayers.

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Main Results:

  • MHS SR vesicles exhibited two to threefold higher Ca2+ efflux rates compared to normal SR.
  • While both normal and MHS channels activated similarly with increasing Ca2+, MHS channels showed prolonged opening below pCa 4.
  • Altered Ca2+ dependence of channel inactivation was observed in MHS SR, even with trans-side Ca2+ changes.

Conclusions:

  • A defect in a low-affinity Ca2+ binding site likely underlies the altered gating of MHS SR channels.
  • This defect may stem from a mutation in the gene encoding the SR calcium release channel.
  • This provides a testable hypothesis for the molecular basis of inherited malignant hyperthermia.