Commentary: extraocular muscle sparing in muscular dystrophy: a critical evaluation of potential protective mechanisms.
Muscles or muscle groups exhibiting responses to neuromuscular disease that are unlike those of other skeletal muscles may provide novel information about pathogenesis leading to improved treatment strategies. The author's laboratory studies the relationship between the unique phenotype of the extraocular muscles and their selective sparing or targeting in neuromuscular disease. This commentary evaluates the evidence for and against four hypotheses for the selective protection of extraocular muscle in Duchenne muscular dystrophy (DMD) and merosin-deficient congenital muscular dystrophy (CMD).
Porter
J D
JD
Department of Anatomy and Neurobiology, University of Kentucky, Lexington 40536-0084, USA. anajohnp@pop.uky.edu
D000222:Adaptation, Physiological / Q000502:physiology; D000818:Animals; D002118:Calcium / Q000378:metabolism; D016166:Free Radical Scavengers / Q000378:metabolism; D006706:Homeostasis / Q000502:physiology; D007797:Laminin / Q000172:deficiency; D008954:Models, Biological; D009137:Muscular Dystrophy, Animal / Q000151:congenital / Q000378:metabolism / Q000503:physiopathology*; D009801:Oculomotor Muscles / Q000502:physiology / Q000503:physiopathology*; D017382:Reactive Oxygen Species / Q000378:metabolism
D016166:Free Radical Scavengers; D007797:Laminin; D017382:Reactive Oxygen Species; D002118:Calcium