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SR function in malignant hyperthermia.

T E Nelson1

  • 1Department of Anesthesiology, University of Texas Health Science Center at Houston.

Cell Calcium
|December 1, 1988
PubMed
Summary
This summary is machine-generated.

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Malignant hyperthermia (MH) is a genetic disorder affecting muscle cell calcium regulation. Research suggests defects in sarcoplasmic reticulum calcium release channels initiate the MH syndrome, not the Ca2+-ATPase protein.

Area of Science:

  • Biochemistry
  • Genetics
  • Physiology

Background:

  • Malignant hyperthermia (MH) is a genetic disorder causing a severe hypermetabolic reaction to anesthetics.
  • A leading hypothesis implicates dysregulation of muscle cell calcium in MH pathogenesis.
  • The sarcoplasmic reticulum (SR) membrane system is the primary regulator of muscle cell calcium.

Purpose of the Study:

  • To review evidence supporting a defect in muscle cell calcium regulation by the SR in MH.
  • To investigate specific SR functions in MH muscle.
  • To identify the primary molecular defect responsible for MH.

Main Methods:

  • In vitro contracture testing of skeletal muscle from MH patients and animals with halothane and caffeine.
  • In vivo and in vitro measurements of muscle cell Ca2+ using microelectrodes and fura-2.

Related Experiment Videos

  • Analysis of calcium-regulating functions in isolated SR from MH muscle, focusing on Ca2+-ATPase and Ca2+ release channels.

    • Main Results:

    • MH muscle exhibits abnormal contracture responses to halothane and caffeine, modifiable by calcium levels.
    • Abnormal resting and stimulated Ca2+ levels were measured in MH muscle cells.
    • The SR Ca2+-ATPase protein was found not to be the cause of MH.
    • Ca2+ release channels and a Ca2+-induced Ca2+ release pathway in the SR are identified as abnormal in MH muscle.

    Conclusions:

    • A defect in muscle cell calcium regulation by the SR is central to MH.
    • The primary defect in MH muscle resides within the SR Ca2+ release channels, not the Ca2+-ATPase.
    • Abnormalities in Ca2+ release pathways are strongly implicated in the MH syndrome.