Journal
BRAIN RESEARCH BULLETIN
Volume 80, Issue 4-5, Pages 282-295Publisher
PERGAMON-ELSEVIER SCIENCE LTD
DOI: 10.1016/j.brainresbull.2009.06.004
Keywords
alpha-Internexin; Intermediate filaments; Neurodegeneration; Neurofilament; Peripherin
Categories
Funding
- Canadian Institutes of Health Research (CIHR)
- ALS Canada
- AFM (Association Francaise contre les Myopathies)
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Intermediate filaments represent the most abundant cytoskeletal element in mature neurons. Mutations and/or accumulations of neuronal intermediate filament proteins are frequently observed in several human neurodegenerative disorders. Although it is now admitted that disorganization of the neurofilament network may be directly involved in neurodegeneration, certain type of perikaryal intermediate filament aggregates confer protection in motor neuron disease. The use of various mouse models provided a better knowledge of the role played by the disorganization of intermediate filaments in the pathogenesis of neurodegenerative disorders, but the mechanisms leading to the formation of these aggregates remain elusive. Here, we will review some neurodegenerative diseases involving intermediate filaments abnormalities and possible mechanisms susceptible to provoke them. (C) 2009 Elsevier Inc. All rights reserved.
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