Dysfunctions of neuronal and glial intermediate filaments in disease
Ronald K.H. Liem, Albee Messing
Ronald K.H. Liem, Albee Messing
Published July 1, 2009
Citation Information: J Clin Invest. 2009;119(7):1814-1824. https://doi.org/10.1172/JCI38003.
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Review Series

Dysfunctions of neuronal and glial intermediate filaments in disease

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 α-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.

Authors

Ronald K.H. Liem, Albee Messing

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Figure 2

Morphological features of glial and neuronal protein aggregates in Alexander disease and CMT.

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Morphological features of glial and neuronal protein aggregates in Alexa...
(A) Rosenthal fibers concentrated in the astrocytic endfeet surrounding a blood vessel (V) in the brain stem of a 1-year-old child with Alexander disease. H&E stain, paraffin section (reproduced with permission from Elsevier [102]). Original magnification, ×62. (B) Rosenthal fibers surrounded by IFs in an astrocyte cell body from a 17-month-old child with Alexander disease, viewed by transmission electron microscopy (reproduced with permission of Wiley-Liss Inc., a subsidiary of John Wiley & Sons Inc. [103]). N, nucleus. (C) Sural nerve biopsy from a CMT patient with an L286P mutations in NFL. The figure shows a giant axon with a cluster of organelles (arrow) and irregular whorls of neurofilaments. (D) Sural nerve biopsy of a CMT patient with an NFL del322C–326N mutation. The figure shows a fiber whose axoplasm consists almost exclusively of microtubules; note the loosening of the external myelin lamellae (panels C and D were reproduced with permission from Brain: a journal of neurology [79]). Scale bars: 1 μm (B); 2 μm (C and D).