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Bunina Bodies in Amyotrophic Lateral Sclerosis
Author(s) -
Okamoto Koichi
Publication year - 1993
Publication title -
neuropathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.701
H-Index - 61
eISSN - 1440-1789
pISSN - 0919-6544
DOI - 10.1111/j.1440-1789.1993.tb00197.x
Subject(s) - golgi apparatus , immunoperoxidase , pathology , neurofilament , amyotrophic lateral sclerosis , immunoelectron microscopy , biology , immunohistochemistry , anatomy , microbiology and biotechnology , endoplasmic reticulum , medicine , antibody , monoclonal antibody , immunology , disease
Bunina bodies (BBs), small eosinophilic intraneuronal inclusions in the remaining lower motor neurons, are the only specific pathological hallmarks of amyotrophic lateral sclerosis (ALS). This report deals with the study of and provides a review on the morphology and morphogenesis of BBs. Immunohistochemical studies disclosed that BBs express cystatin C, but they failed to react with a variety of other antibodies. Serial electron microscopic investigations documented that BBs consisted of electron‐dense amorphous material that contains tubular and vesicular structures and that in the early stages of BB formation the amorphous material was sometimes present around the tubular and vesicular structures. From immunoelectron microscopic studies with the antiserum to cystatin C, there was evidence for the presence of immunoperoxidase reaction products in the periphery of BBs. Furthermore, cystatin C was mainly localized in the Golgi apparatus of anterior horn cells of an adult cat. It would appear that BBs may represent abnormal accumulations of unknown proteinaceous materials that are associated with the Golgi apparatus.

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