Figure 2
Morphological features of glial and neuronal protein aggregates in Alexander disease and CMT.
(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).
