Intense Superoxide Dismutase-1 Immunoreactivity in Intracytoplasmic Hyaline Inclusions of Familial Amyotrophic Lateral Sclerosis with Posterior Column Involvement (original) (raw)
Journal Article
,
Department of Pathology,
Tokyo Women's Medical College
, 8-1 Kawada-cho, Shinjuku-ku, Tokyo 162, Japan
Correspondence to: Noriyuki Shibata, MD, Department of Pathology, Tokyo Women's Medical College, 8-1 Kawada-cho, Shinjuku-ku, Tokyo 162, Japan.
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Division of Neuropathology, Department of Pathology,
Montefiore Medical Center
, 111 East 210th Street, Bronx, NY 10467-2490
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Department of Pathology,
Tokyo Women's Medical College
, 8-1 Kawada-cho, Shinjuku-ku, Tokyo 162, Japan
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Department of Neurology,
Northwestern University Medical School
, Chicago, IL 60611-3008
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Department of Neurology,
Northwestern University Medical School
, Chicago, IL 60611-3008
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Department of Neurology,
Northwestern University Medical School
, Chicago, IL 60611-3008
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Department of Internal Medicine,
Yamagata University School of Medicine
, 2-2-2 Iidanishi, Yamagata City, Yamagata 990–23, Japan
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Department of Pediatrics,
Yamanashi Medical College
, 1110 Shimokato, Tamaho-cho, Nakakoma-gun, Yamanashi 409-38, Japan
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PDF Cite
Noriyuki Shibata, Asao Hirano, Makio Kobayashi, Teepu Siddique, Han-Xiang Deng, Wu-Yen Hung, Takeo Kato, Kohtaro Asayama, Intense Superoxide Dismutase-1 Immunoreactivity in Intracytoplasmic Hyaline Inclusions of Familial Amyotrophic Lateral Sclerosis with Posterior Column Involvement, Journal of Neuropathology & Experimental Neurology, Volume 55, Issue 4, April 1996, Pages 481–490, https://doi.org/10.1097/00005072-199604000-00011
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Abstract
This report concerns retrospective immunohistochemical and immunoelectron microscopic studies on superoxide dismutase-1 (SOD1) in intracytoplasmic hyaline inclusions (IHIs) of the anterior horn cells of three patients with familial amyotrophic lateral sclerosis (ALS) with posterior column involvement. All of the patients were members of the American “C” family. Almost all of the IHIs, present in the soma and cordlike swollen neurites of some affected neurons of the three patients, were intensely stained by an antibody to human SOD1. By contrast, the cytoplasm of anterior horn cells of the ALS patients and of ten control individuals reacted only weakly with the antibody or not at all. Immunoelectron microscopy revealed that the granule-associated thick linear structures that composed the IHIs were intensely labeled by the antibody to SOD1. The IHIs were also positively stained by antibodies to ubiquitin and phosphorylated neurofilament protein, with the distribution of immunoreactivity resembling that seen with the anti-SOD1 antibody. The DNA analysis disclosed a single-site GCC to GTC substitution at codon 4 (Ala4 → Val) in the SOD1 gene from the brain samples of the patients and from the peripheral blood of their family members. Our results suggest that SOD1 is a component of IHIs and may interact with ubiquitin and neurofilament protein, and point to the possibility that the presence of intense SOD1 immunoreactivity in the IHIs may be of relevance in processes involving structurally altered SOD1 molecules encoded by the mutated gene.
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© 1996 by the American Association of Neuropathologists
Topic:
- mutation
- hyaline
- anterior horn cells
- codon nucleotides
- cytoplasm
- genes
- microscopy, immunoelectron
- neurites
- neurofilament proteins
- neurons
- ubiquitin
- antibodies
- brain
- dna analysis
- dorsal column
- sod1 gene
- granules
- molecule
- superoxide dismutase 1
- amyotrophic lateral sclerosis 1
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