Disturbed Ca2+ Homeostasis in Muscle-Wasting Disorders

Adv Exp Med Biol. 2018:1088:307-326. doi: 10.1007/978-981-13-1435-3_14.

Abstract

Ca2+ is essential for proper structure and function of skeletal muscle. It not only activates contraction and force development but also participates in multiple signaling pathways. Low levels of Ca2+ restrain muscle regeneration by limiting the fusion of satellite cells. Ironically, sustained elevations of Ca2+ also result in muscle degeneration as this ion promotes high rates of protein breakdown. Moreover, transforming growth factors (TGFs) which are well known for controlling muscle growth also regulate Ca2+ channels. Thus, therapies focused on changing levels of Ca2+ and TGFs are promising for treating muscle-wasting disorders. Three principal systems govern the homeostasis of Ca2+, namely, excitation-contraction (EC) coupling, excitation-coupled Ca2+ entry (ECCE), and store-operated Ca2+ entry (SOCE). Accordingly, alterations in these systems can lead to weakness and atrophy in many hereditary diseases, such as Brody disease, central core disease (CCD), tubular aggregate myopathy (TAM), myotonic dystrophy type 1 (MD1), oculopharyngeal muscular dystrophy (OPMD), and Duchenne muscular dystrophy (DMD). Here, the interrelationship between all these molecules and processes is reviewed.

Keywords: Atrophy; Ca2+ channel; EC coupling; Intracellular Ca2+; Myogenesis.

Publication types

  • Review

MeSH terms

  • Calcium / physiology*
  • Calcium Signaling*
  • Excitation Contraction Coupling
  • Homeostasis*
  • Humans
  • Muscle, Skeletal / physiopathology*
  • Muscular Atrophy / physiopathology*
  • Muscular Diseases / genetics
  • Muscular Diseases / physiopathology

Substances

  • Calcium