Calcium Misregulation and the Pathogenesis of Muscular Dystrophy

  • F.W. HOPF
  • P.R. TURNER
  • R.A. STEINHARDT
Part of the Subcellular Biochemistry book series (SCBI, volume 45)

Abstract

Although the exact nature of the relationship between calcium and the pathogenesis of Duchenne muscular dystrophy (DMD) is not fully understood, this is an important issue which has been addressed in several recent reviews (Alderton and Steinhardt, 2000a, Gailly, 2002, Allen et al., 2005). A key question when trying to understand the cellular basis of DMD is how the absence or low level of expression of dystrophin, a cytoskeletal protein, results in the slow but progressive necrosis of muscle fibres. Although loss of cytoskeletal and sarcolemmal integrity which results from the absence of dystrophin clearly plays a key role in the pathogenesis associated with DMD, a number of lines of evidence also establish a role for misregulation of calcium ions in the DMD pathology, particularly in the cytoplasmic space just under the sarcolemma. A number of calcium-permeable channels have been identified which can exhibit greater activity in dystrophic muscle cells, and existing evidence suggests that these may represent different variants of the same channel type (perhaps the transient receptor potential channel, TRPC). In addition, a prominent role for calcium-activated proteases in the DMD pathology has been established, as well as modulation of other intracellular regulatory proteins and signaling pathways. Whether dystrophin and its associated proteins have a direct role in the regulation of calcium ions, calcium channels or intracellular calcium stores, or indirectly alters calcium regulation through enhancement of membrane tearing, remains unclear. Here we focus on areas of consensus or divergence amongst the existing literature, and propose areas where future research would be especially valuable

Keywords

dystrophin-glycoprotein complex sarcolemma membrane tears calcium channels calcium-activated proteases 

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Reference

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Copyright information

© Springer 2007

Authors and Affiliations

  • F.W. HOPF
    • 1
  • P.R. TURNER
    • 2
  • R.A. STEINHARDT
    • 3
  1. 1.Ernest Gallo Clinic and Research CenterUniversity of California, San FranciscoSuite 200, EmeryvilleU.S.A
  2. 2.Panomics6519 Dumbarton CircleU.S.A
  3. 3.Department of Molecular and Cell biologyUniversity of CaliforniaBerkeleyU.S.A

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