Neurofilaments and neurological disease

Neurofilaments are one of the major components of the neuronal cytoskeleton and are responsible for maintaining the calibre of axons. They are modified by post‐translational changes that are regulated in complex fashions including by the interaction with neighbouring glial cells. Neurofilament accum...

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Bibliographic Details
Published in:BioEssays 2003-04, Vol.25 (4), p.346-355
Main Authors: Al-Chalabi, Ammar, Miller, Christopher C. J.
Format: Article
Language:English
Subjects:
Animals
Biological Transport - physiology
Humans
Intermediate Filaments - metabolism
Intermediate Filaments - ultrastructure
Mice
Mice, Transgenic
Mutation
Nervous System Diseases - genetics
Nervous System Diseases - metabolism
Nervous System Diseases - pathology
Neurofilament Proteins - chemistry
Neurofilament Proteins - genetics
Neurofilament Proteins - metabolism
Neurons - metabolism
Neurons - ultrastructure
Protein Subunits - metabolism
Spinal Cord - metabolism
Spinal Cord - pathology
Superoxide Dismutase - genetics
Superoxide Dismutase-1
tau Proteins - genetics
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Summary:Neurofilaments are one of the major components of the neuronal cytoskeleton and are responsible for maintaining the calibre of axons. They are modified by post‐translational changes that are regulated in complex fashions including by the interaction with neighbouring glial cells. Neurofilament accumulations are seen in several neurological diseases and neurofilament mutations have now been associated with Charcot‐Marie‐Tooth disease, Parkinson's disease and amyotrophic lateral sclerosis. In this review, we discuss the structure, normal function and molecular pathology of neurofilaments. BioEssays 25:346–355, 2003. © 2003 Wiley Periodicals, Inc.
ISSN:0265-9247
1521-1878
DOI:10.1002/bies.10251