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題名 | Plasmapheresis Based on Molecular Immunology in Myasthenias= |
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作者 | Takamori, Masaharu; Maruta, Takahiro; |
期刊 | Acta Neurologica Taiwanica |
出版日期 | 20001200 |
卷期 | 9:4 民89.12 |
頁次 | 頁273-281 |
分類號 | 415.9413 |
語文 | eng |
關鍵詞 | 重症肌無力症; 血漿分離術; Myasthenia gravis; Lambert-eaton myasthenic syndrome; Acetylcholine receptor; Ryanodine receptor; Voltage-gated calcium channel; Autoimmunity; Plasmapheresis; |
英文摘要 | In myasthenia gravis, current work based on the molecular information about acetylcholine receptor (AChR) has contributed to the understanding of the possible localization of myasthenic domains on AChR, such as sites recognized by the antibodies which cause the acceleration of AChR degradation and the complement-mediated lysis of postsynaptic membrane (residues 125-147 and 67-76 of the AChR α-subunit), and the Ach-binding site recognized by the antibodies causing the blockade of Ach-binding to AChR (residues 183-200 of the AChR α-subunit) (termed as blocking antibodies). Synthetic peptide technology offers a means to define antigenic structure of AChR and devises selective removal of pathogenic antibodies from blood circulation with minimal side-effects. First, we synthesized the peptide corresponding to α183-200, this was immunologically estimated as a myasthenogenic antigen in animals; the result was then to make use of antigen-specific therapy in human myasthenia gravis. Twenty-two myasthenic patients who were positive for anti-peptide (α183-200) and anti-AChR blocking antibodies were treated by purifying the plasma with a peptide-bound adsorbent. From the results of 77 trials (1-4 trials per patient), a greater then 50% reduction of the blocking antibodies was seen in 69% of trials, and the clinical efficacy was obtained in 68% of trials. The total IgG was minimally reduced by this treatment, suggesting that the present treatment offers an advantage over the means of plasmapheresis which tends to remove other plasma constituents that are clinically important. In many of thymoma-associated myasthenia gravis patients, we have detected antibodies to ryanodine receptor that is a food protein in the junctional gap between the sarcoplasmic reticulum and the calcium channel-containing T-tubules in skeletal muscle that functions as a calcium-release channel during E-C coupling. The immunohistochemical study demonstrated this receptor protein in thymoma cells; the electrophysiological study showed muscle contraction block in addition to transmission block in such patients whose sera contains anti-ryanodine receptor antibodies as well as anti-AChR antibodies. At the time of myasthenic crisis, anti-ryanodine receptor antibody titers had a tendancy to elevate, and the plasmapheresis gave relief to crisis. In Lambert-Eaton myasthenic syndrome, antibodies to voltage-gated calcium channel (P/Q-type) in the nerve terminal play a crucial role in causing deficient Ach release, resulting in myasthenic weakness. Our study unveiled 3 pathogenic domains in the molecular structure of α1-subunit of this channel protein. Plasmapheresis relieved patients from muscle weakness. |
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