Morphological Aspects of Superoxide Dismutase-1 Mutation in Amyotrophic Lateral Sclerosis and its Transgenic Mouse Model (Symposium VIIHistochemistry for Neuroscience Research)
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概要
- 論文の詳細を見る
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease that primarily involves the motor neuron system. Recent investigations have obtained evidence that mutations in the gene for superoxide dismutase-1 (SOD1) are detected in a subset of familial ALS patients, and that mutant SOD1-expressing transgenic mice develop ALS-like clinicopathological features. Several in vitro studies suggest that SOD1-mutated ALS is caused by a newly acquired neurotoxicity of mutant SOD1, but not by reduced enzyme activity. From the viewpoint of morphology, we analyzed the immunolocalization of SOD1 and some other substances in spinal cords from familial ALS patients with SOD1 Ala^4→Val mutation. The spinal cords of the ALS patients demonstrated the characteristic neuronal hyaline inclusions (NHIs) immunoreactive with antibodies to ubiquitin and phosphorylated neurofilament protein (NFP) in the lower motor neurons and cordlike swollen axons. The NHIs contained the epitopes of SOD1 and N^ε-carboxymethyllysine (CML), one of the major advanced glycation endproducts (AGE), whereas there was no focal accumulation of SOD1 and CML in control individuals. Immunoelectron microscopy depicted the SODl and CML determinants on the granule-associated thick linear structures that mainly compose the NHIs. We also performed a similar study on mice carrying a transgene for Gly^93→Ala mutant SOD1. The spinal cords of the transgenic mice were characterized by the appearance of NHIs resembling those of familial ALS and by vacuolar degeneration. The mouse NHIs were immunoreactive for ubiquitin, phophorylated NFP, SOD1 and CML. Our findings of the coexistence of SOD1 and AGE in both the human and mouse NHIs indicate that certain substances are implicated in glycoxidation in the presence of oxidative stress originating from mutant SOD1 and finally deposited in the NHIs, suggesting a pathogenic role of the oxidative processes in motor neuron degeneration in vivo.
- 日本組織細胞化学会の論文
著者
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Shibata Noriyuki
Department of Pathology, Tokyo Women’s Medical University
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Kobayashi Makio
Department of Pathology, Tokyo Women’s Medical University
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Dal Canto
Department Of Pathology North-western University Medical School
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Dal Canto
Department Of Pathology Northwestern University Medical School
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ASAYAMA KOHTARO
Department of Pediatrics, School of Medicine, University of Occupational and Environmental Health
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HORIUCHI Seiko
Department of Dentistry, Toranomon Hospital
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HIRANO Asao
Division of Neuropathology, Montefiore Medical Center
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Shibata Noriyuki
Department Of Pathology Tokyo Women's Medical University
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Shibata Noriyuki
Dept. Of Pathology Tokyo Women's Medical University
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Shibata Noriyuki
Department Of Pathology Tokyo Women's Medical College
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Sakayori Noriko
Department Of Pathology Tokyo Women's Medical University
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Kobayashi Masatoshi
Department Of Anatomy & Histology Kurume University School Of Medicine
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Kobayashi Makio
Department Of Pathology Tokyo Women's Medical College
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Kobayashi Makio
Dept. Of Pathology Tokyo Women's Medical University
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Hirano Asao
Division Of Neuropathology And Department Of Pathology Montefiore Medical Center/albert Einstein Col
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Saito N
Department Of Molecular And Pharmacological Neuroscience Graduate School Of Biomedical Sciences Hiro
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Kobayashi M
Departments Of Pathology Tokyo Women
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Gurney Mark
Cns Diseases Research Unit Pharmacia And Upjohn Inc.
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Horiuchi Seiko
Department Of Biochemistry Kumamoto University School Of Medicine
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Asayama Kohtaro
Department Of Pediatrics School Of Medicine University Of Occupational And Environmental Health
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Hirano A
Division Of Neuropathology Montefiore Medical Center
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Kobayashi M
Aska Pharmaceutical Co. Ltd. Research & Development Division
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Kobayashi Makio
Department Of Immunology And Pathology Yamanashi Medical College
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Shibata Noriyuki
Department Of Pathology School Of Medicine Tokyo Women's Medical University
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Kobayashi Makio
Department of Agricultural Chemistry, Faculty of Agriculture, Hokkaido University
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