Abstract
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.
Copyright 2003 Wiley Periodicals, Inc.
MeSH terms
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Animals
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Biological Transport / physiology
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Humans
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Intermediate Filaments / metabolism*
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Intermediate Filaments / ultrastructure
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Mice
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Mice, Transgenic
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Mutation
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Nervous System Diseases / genetics
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Nervous System Diseases / metabolism*
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Nervous System Diseases / pathology
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Neurofilament Proteins / chemistry
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Neurofilament Proteins / genetics
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Neurofilament Proteins / metabolism*
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Neurons / metabolism
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Neurons / ultrastructure
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Protein Subunits / metabolism
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Spinal Cord / metabolism
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Spinal Cord / pathology
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Superoxide Dismutase / genetics
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Superoxide Dismutase-1
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tau Proteins / genetics
Substances
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Neurofilament Proteins
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Protein Subunits
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SOD1 protein, human
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tau Proteins
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Sod1 protein, mouse
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Superoxide Dismutase
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Superoxide Dismutase-1