Calcium misregulation and the pathogenesis of muscular dystrophy

Sub-cellular Biochemistry
F W HopfR A Steinhardt

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 modu...Continue Reading

Citations

Jan 28, 2009·Annals of Medicine·Adrian WaiteDerek J Blake
Feb 7, 2012·Archivum Immunologiae Et Therapiae Experimentalis·Alexander SpassovChristiane Kunert-Keil
Jan 3, 2013·Journal of Pineal Research·Jae-Hyung ParkDae-Kyu Song
Feb 22, 2014·Human Molecular Genetics·Sanjeewa A GoonasekeraJeffery D Molkentin
Jun 16, 2017·Annals of Noninvasive Electrocardiology : the Official Journal of the International Society for Holter and Noninvasive Electrocardiology, Inc·Nikolaos FragakisVassilios Vassilikos
Jul 15, 2009·Journal of Cardiovascular Pharmacology·Jonathan A StiberPaul B Rosenberg
Oct 30, 2010·Experimental Animals·Hiroyuki SorimachiYasuko Ono
Mar 16, 2011·Muscle & Nerve·Chad D MarkertRobert W Grange
Apr 14, 2011·Journal of Pineal Research·Youssef HibaouiOlivier M Dorchies
Feb 6, 2015·American Journal of Physiology. Cell Physiology·Davi A G MázalaEva R Chin
Oct 23, 2012·Journal of Applied Physiology·Yu WangShankar Subramaniam
Aug 1, 2019·American Journal of Physiology. Cell Physiology·Nandita NiranjanGopal J Babu
Oct 9, 2009·The Journal of Biological Chemistry·Jessica SabourinBruno Constantin
Nov 28, 2012·PloS One·Xiaoli ZhaoNoah Weisleder
Dec 12, 2012·Journal of Applied Physiology·Chady H Hakim, Dongsheng Duan
Aug 5, 2011·American Journal of Cardiovascular Drugs : Drugs, Devices, and Other Interventions·Daniel P JudgeKathryn R Wagner
Oct 21, 2011·Journal of Biomedicine & Biotechnology·David S Gokhin, Velia M Fowler
Apr 27, 2013·Skeletal Muscle·James P EnnenAtsushi Asakura
Jan 9, 2013·Molecular & Cellular Proteomics : MCP·Sree RayavarapuKanneboyina Nagaraju
Jul 26, 2017·Microscopy Research and Technique·Sara SalucciSabrina Burattini
Mar 20, 2019·Journal of Muscle Research and Cell Motility·Sandra MurphyKay Ohlendieck
Dec 24, 2011·The American Journal of Pathology·Julie Reutenauer-PatteOlivier M Dorchies
Apr 8, 2021·Cellular and Molecular Life Sciences : CMLS·Alicja Starosta, Patryk Konieczny
Sep 24, 2021·Pflügers Archiv : European journal of physiology·Kay Ohlendieck, Dieter Swandulla

❮ Previous
Next ❯

Related Concepts

Related Feeds

Calcium & Bioenergetics

Bioenergetic processes, including cellular respiration and photosynthesis, concern the transformation of energy by cells. Here is the latest research on the role of calcium in bioenergetics.