Neuronal intermediate filaments and neurodegenerative disorders.

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.