Authors: Fujita, Kengo¹; Ito, Hidefumi²; Nakano, Satoshi¹; Kinoshita, Yoshimi¹; Wate, Reika¹; Kusaka, Hirofumi¹

Source: Acta Neuropathologica, Volume 116, Number 4, October 2008 , pp. 439-445(7)

Publisher: Springer

Abstract:

This report concerns an immunohistochemical investigation on RNA-related proteins in the basophilic inclusions (BIs) from patients with adult-onset atypical motor neuron disease. Formalin-fixed, paraffin-embedded sections of the motor cortex and the lumbar spinal cord were examined. The BIs appeared blue in color with H&E and Nissl stain, and pink with methylgreen-pyronin stain. Ribonuclease pretreatment abolished the methylgreen-pyronin staining, suggesting that the BIs contained RNA. Immunohistochemically, the BIs were distinctly labeled with the antibodies against poly(A)-binding protein 1, T cell intracellular antigen 1, and ribosomal protein S6. These proteins are essential constituents of stress granules. In contrast, the BIs were not immunoreactive for ribosomal protein L28 and decapping enzyme 1, which are core components of transport ribonucleoprotein particles and processing bodies, respectively. Moreover, the BIs were not immunopositive for TDP-43. Our results imply that translation attenuation could be involved in the processes of BI formation in this disorder.

Keywords: Motor neuron disease; Basophilic inclusion; RNA-binding protein; Stress granule; Poly(A)-binding protein; T cell intracellular antigen 1

Document Type: Research article

DOI: http://dx.doi.org/10.1007/s00401-008-0415-x

Affiliations: 1: Department of Neurology, Kansai Medical University, 10-15, Fumizono-cho, Moriguchi, Osaka, 570-8507, Japan 2: Department of Neurology, Kansai Medical University, 10-15, Fumizono-cho, Moriguchi, Osaka, 570-8507, Japan, Email: itoh@takii.kmu.ac.jp

Publication date: 2008-10-01

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• In this Subject: Neurology & Psychiatry ,  Pathology
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