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Purification and Transplantation of Myogenic Progenitor Cell Derived Exosomes to Improve Cardiac Function in Duchenne Muscular Dystrophic Mice
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Abnormal Calcium Handling in Duchenne Muscular Dystrophy: Mechanisms and Potential Therapies

Satvik Mareedu1, Emily D Million2, Dongsheng Duan2,3

  • 1Department of Cell Biology and Molecular Medicine, New Jersey Medical School, Rutgers University, Newark, NJ, United States.

Frontiers in Physiology
|April 26, 2021
PubMed

Insights

Duchenne muscular dystrophy (DMD) involves elevated intracellular calcium, damaging muscle. Targeting calcium regulation offers a promising therapeutic strategy for this genetic muscle-wasting disease.

Area of Science:

  • Biochemistry
  • Cell Biology
  • Genetics

Background:

  • Duchenne muscular dystrophy (DMD) is a severe X-linked muscle-wasting disease caused by dystrophin deficiency.
  • DMD leads to progressive muscle degeneration, necrosis, inflammation, fibrosis, and ultimately respiratory and cardiac failure.
  • Currently, no curative treatments exist for DMD, highlighting the need for novel therapeutic strategies.

Purpose of the Study:

  • To provide an updated overview of the mechanistic roles of cellular calcium handling in DMD pathogenesis.
  • To explore how sarcolemma, sarcoplasmic/endoplasmic reticulum, and mitochondria contribute to elevated intracellular calcium in DMD.
  • To discuss current therapeutic approaches targeting calcium homeostasis for DMD treatment.

Main Methods:

  • Review of existing literature on calcium dysregulation in Duchenne muscular dystrophy.
  • Analysis of the roles of key cellular organelles (sarcolemma, SR/ER, mitochondria) in calcium homeostasis.
  • Synthesis of current research on therapeutic strategies aimed at restoring calcium balance.

Main Results:

  • Dystrophin deficiency in DMD leads to abnormal and sustained elevation of intracellular calcium levels.
  • The sarcolemma, sarcoplasmic/endoplasmic reticulum, and mitochondria are all implicated in this calcium dysregulation.
  • These calcium handling abnormalities significantly contribute to the pathogenesis and progression of DMD.

Conclusions:

  • Restoring calcium homeostasis represents a promising therapeutic avenue for Duchenne muscular dystrophy.
  • Targeting specific calcium-handling proteins and mechanisms holds potential for novel DMD treatments.
  • Further research into cellular calcium regulation is crucial for developing effective therapies for DMD.
Keywords:
Duchenne muscular dystrophycalciumdystrophinmitochondriaryanodine receptorsarco(endo)plasmic reticulum calcium ATPasesarcolemmasarcolipin

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