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Related Experiment Videos

Ca2+ signalling and muscle disease.

D H MacLennan1

  • 1Banting and Best Department of Medical Research, University of Toronto, Ontario, Canada. david.maclennan@utoronto.ca

European Journal of Biochemistry
|August 22, 2000
PubMed
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Calcium (Ca2+) signaling is vital for cell function but prolonged elevation causes cell death. Muscle Ca2+ regulation proteins, when abnormal, lead to diseases like malignant hyperthermia and myopathies.

Area of Science:

  • Cellular Biology
  • Molecular Physiology
  • Biochemistry

Background:

  • Transient intracellular calcium (Ca2+) elevations are crucial signaling events for numerous cellular processes.
  • Prolonged or excessive Ca2+ levels (>10 microM) are detrimental, potentially inducing apoptosis.
  • Muscle Ca2+ dysregulation, due to protein abnormalities, can cause specific diseases rather than cell death.

Purpose of the Study:

  • To review the key Ca2+ regulatory proteins in muscle.
  • To highlight the role of Ca2+ channel and pump abnormalities in muscle diseases.
  • To discuss the impact of Ca2+ binding proteins on Ca2+ storage and cellular function.

Main Methods:

  • Review of literature on Ca2+ signaling and muscle physiology.
  • Analysis of studies involving knockout and transgenic mouse models.

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  • Examination of genetic and functional studies of Ca2+ regulatory proteins.

    • Main Results:

    • Abnormalities in L-type Ca2+ channels and Ca2+ release channels are implicated in malignant hyperthermia and central core disease.
    • Defects in Sarco(endo)plasmic reticulum Ca2+ ATPases (SERCAs), like SERCA1a, cause impaired muscle relaxation (Brody disease).
    • Modulation of SERCA activity by phospholamban and sarcolipin affects cardiac contractility and can contribute to cardiomyopathies.
    • Altered expression of Ca2+ binding proteins (calsequestrin, calreticulin) impacts cardiac development and function, leading to cardiomyopathy.

    Conclusions:

    • Specific Ca2+ regulatory proteins are critical for maintaining muscle function and preventing disease.
    • Dysregulation of Ca2+ channels, pumps, and binding proteins underlies various muscle disorders.
    • Further research into these proteins offers potential therapeutic targets for muscle diseases.