Journal
JOURNAL OF THE NEUROLOGICAL SCIENCES
Volume 302, Issue 1-2, Pages 14-18Publisher
ELSEVIER
DOI: 10.1016/j.jns.2010.12.023
Keywords
Amyotrophic lateral sclerosis; Bunina bodies; Lewy body-like inclusions; Peripherin
Categories
Funding
- Japan Society for the Promotion of Science
- Ministry of Health, Labour and Welfare of Japan
- Ministry of Education, Culture, Sports, Science and Technology of Japan
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Peripherin is a type III intermediate filament protein expressed with low levels in spinal motor neurons. Amyotrophic lateral sclerosis (ALS) is characterized by the presence of Bunina bodies, skein-like inclusions, and Lewy body-like inclusions (LBLIs) in the remaining anterior horn cells, where the first and third structures are detected by Hematoxylin-Eosin (H & E) staining. We examined paraffin sections of lumbar spinal cords from six ALS patients, using H & E staining and immunostaining for human peripherin. The results demonstrated that there were a total of 73 anterior horn cells containing one or more Bunina bodies, and that twelve of these cells (approximately 16.4%) demonstrated peripherin-positive Bunina bodies. In fact, some part of chain-like Bunina bodies showed peripherin-positive reaction, although there were a much higher number of non-immunoreacitive Bunina bodies in each neuron. LBLIs were clearly immunostained for peripherin corresponding to the core, while some of them showed different types of immunoreactivities due to oblique cutting of inclusions. Our findings suggest that although the mechanisms underlying peripherin co-localization in Bunina bodies are unknown, peripherin could be involved in forming these inclusions. Furthermore, following cystatin C and transferrin, peripherin is the third most prevalent protein that partially localizes in Bunina bodies. (C) 2010 Elsevier B.V. All rights reserved.
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