Journal
MUSCLE & NERVE
Volume 32, Issue 6, Pages 693-709Publisher
WILEY
DOI: 10.1002/mus.20373
Keywords
amyotrophic lateral sclerosis; cell injury; chaperones; heat shock proteins; inflammatory myopathy; motor neuron disease; muscle; muscular dystrophy; neuromuscular diseases; neuropathy; stress proteins
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The heat shock proteins are families of proteins with known activities that include chaperoning nascent peptides within the cell and cytoprotection. Most work on the nervous system has related to the role of heat shock proteins in neuroprotection from either hypoxic-ischemic or traumatic injury. The role of these proteins during normal physiological activity and injury is still under investigation. Heat shock proteins in neuromuscular disease have been investigated to some extent but were largely neglected until recently. The goal of this review is to summarize the evidence linking heat shock proteins with neuromuscular disease and to provide some insight into the roles or functions of these proteins in disease states.
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