Dysfunctions of neuronal and glial intermediate filaments in disease

J Clin Invest. 2009 Jul;119(7):1814-24. doi: 10.1172/JCI38003. Epub 2009 Jul 1.

Abstract

Intermediate filaments (IFs) are abundant structures found in most eukaryotic cells, including those in the nervous system. In the CNS, the primary components of neuronal IFs are alpha-internexin and the neurofilament triplet proteins. In the peripheral nervous system, a fifth neuronal IF protein known as peripherin is also present. IFs in astrocytes are primarily composed of glial fibrillary acidic protein (GFAP), although vimentin is also expressed in immature astrocytes and some mature astrocytes. In this Review, we focus on the IFs of glial cells (primarily GFAP) and neurons as well as their relationship to different neurodegenerative diseases.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Alexander Disease / etiology
  • Amyotrophic Lateral Sclerosis / etiology
  • Animals
  • Astrocytes / physiology
  • Charcot-Marie-Tooth Disease / etiology
  • Cytomegalovirus Infections / etiology
  • Cytoskeletal Proteins / physiology
  • Glial Fibrillary Acidic Protein / cerebrospinal fluid
  • Glial Fibrillary Acidic Protein / physiology
  • Humans
  • Intermediate Filaments / physiology*
  • Neurodegenerative Diseases / etiology*
  • Neurofilament Proteins / genetics
  • Neuroglia / physiology*
  • Neurons / physiology*
  • Protein Tyrosine Phosphatases, Non-Receptor / genetics

Substances

  • Cytoskeletal Proteins
  • GAN protein, human
  • Glial Fibrillary Acidic Protein
  • Neurofilament Proteins
  • neurofilament protein L
  • MTMR2 protein, human
  • Protein Tyrosine Phosphatases, Non-Receptor