Abstract
Sera from 26% of patients with sporadic amyotrophic lateral sclerosis (ALS) induced in vitro apoptosis of a human neuroblastoma cell line, as detected by two methods, and most contained anti-Fas autoantibodies. In contrast, Alzheimer sera (studied as controls) very rarely induced apoptosis and did not contain detectable anti-Fas antibodies. Soluble Fas-ligand levels in ALS sera were not different from those in normal sera, except for slightly higher levels in a single case. In mixed cultures of rat embryonic brain and spinal cord cells, ALS sera (and agonistic anti-Fas monoclonal antibodies and soluble Fas-ligand) induced the apoptosis of a subpopulation of neurons. These neurons were motoneurons on the basis of staining with the monoclonal antibody SMI 32 and Fas expression was restricted to these SMI 32-positive neurons. These data are compatible with the hypothesis of the participation of an autoimmune mechanism possibly related to anti-Fas autoantibodies in certain ALS patients.
Publication types
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Research Support, Non-U.S. Gov't
MeSH terms
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Amyotrophic Lateral Sclerosis / immunology*
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Animals
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Antibodies, Monoclonal / pharmacology
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Apoptosis / drug effects
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Apoptosis / immunology*
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Autoantibodies / analysis
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Autoantibodies / pharmacology
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Blotting, Western
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Caspase 3
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Caspase 8
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Caspase 9
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Caspase Inhibitors
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Central Nervous System / cytology
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Enzyme Inhibitors / pharmacology
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Enzyme-Linked Immunosorbent Assay
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Female
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Fluorescent Antibody Technique, Indirect
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Humans
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Immune Sera / pharmacology
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In Situ Nick-End Labeling
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In Vitro Techniques
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Jurkat Cells
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Male
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Middle Aged
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Motor Neurons / cytology*
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Motor Neurons / immunology*
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Neuroblastoma
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Rats
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Recombinant Proteins / immunology
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Tumor Cells, Cultured
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fas Receptor / immunology*
Substances
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Antibodies, Monoclonal
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Autoantibodies
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Caspase Inhibitors
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Enzyme Inhibitors
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Immune Sera
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Recombinant Proteins
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fas Receptor
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CASP3 protein, human
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CASP8 protein, human
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CASP9 protein, human
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Casp3 protein, rat
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Casp8 protein, rat
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Casp9 protein, rat
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Caspase 3
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Caspase 8
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Caspase 9