Neuronal intermediate filaments

Michael K. Lee, Don W. Cleveland

Research output: Contribution to journalReview articlepeer-review

359 Scopus citations

Abstract

Neurofilaments (NFs) are the most abundant structural components in large- diameter myelinated axons. Assembled as obligate heteropolymers requiring NF- L and substoichiometric amounts of NF-M and/or NF-H, NF investment into axons is essential for establishment of axonal caliber, itself a key determinant of conduction velocity. Use of transgenic mice to increase axonal accumulation of NFs or to express mutant NFs subunits has proven that aberrant organization or assembly of NFs is sufficient to cause disease arising from selective dysfunction and degeneration of motor neurons. Because aberrant accumulation of NFs is a common pathology in a series of motor neuron diseases-including amyotrophic lateral sclerosis-NF misaccumulation, and the resultant disruption in axonal transport, is probably a key intermediate in the pathogenesis of these diseases.

Original languageEnglish (US)
Pages (from-to)187-217
Number of pages31
JournalAnnual review of neuroscience
Volume19
DOIs
StatePublished - 1996

Keywords

  • axonal cytoskeleton
  • axonal transport
  • motor neuron disease
  • neurofilaments
  • radial growth

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