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TRAPSの診断と新しい治療法の展開
http://hdl.handle.net/10069/24070
http://hdl.handle.net/10069/2407043032865-9715-4705-8c15-56e511c9d3ee
名前 / ファイル | ライセンス | アクション |
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JJCI30_90.pdf (1.2 MB)
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Item type | 学術雑誌論文 / Journal Article(1) | |||||
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公開日 | 2010-10-05 | |||||
タイトル | ||||||
タイトル | TRAPSの診断と新しい治療法の展開 | |||||
言語 | ||||||
言語 | jpn | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | Autoinflammatory syndrome | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | TNF-associated periodic syndrome | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | TRAPS | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | TNF | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | TNFRSF1A | |||||
資源タイプ | ||||||
資源タイプ識別子 | http://purl.org/coar/resource_type/c_6501 | |||||
資源タイプ | journal article | |||||
著者 |
井田, 弘明
× 井田, 弘明× 江口, 勝美 |
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著者別名 | ||||||
姓名 | Ida, Hiroaki | |||||
著者別名 | ||||||
姓名 | Eguchi, Katsumi | |||||
その他のタイトル | ||||||
その他のタイトル | TNF receptor-associated periodic syndrome (TRAPS) in Japan: clinical characterization, pathogenesis, diagnostic criteria, and treatment | |||||
抄録 | ||||||
内容記述タイプ | Abstract | |||||
内容記述 | TNF-associated periodic syndrome (TRAPS)は,TNFが病態の中心と考えられる遺伝性周期性発熱症候群の一つである.TNFRSF1A(TNFR1)分子が細胞表面に留まり,TNFからの反応が持続するため,発熱などの様々なTRAPS症状が出現すると単純に考えられてきた.ところが,最近,TNFRSF1A分子の切断異常がみられない症例や突然変異のないTRAPS症例もあること,さらに孤発例も存在することが判明し,TRAPSとは大変heterogeneousな症候群であることがわかってきた.最近,細胞表面に発現されないTNFRSF1A分子が,TNFと無関係に細胞内で凝集し,NF-κBの活性化やアポトーシス誘導を生じていることも報告され,TRAPSの病因は混沌としている.本邦において,現在までTNFRSF1A遺伝子に突然変異をもつTRAPS症例は5家系15名と少ないが,突然変異のない孤発例は多い.本稿では,TRAPSについて自験例を提示しながら臨床像を紹介するとともに,TNFRSF1A分子の発現制御機構から考えられるTRAPSの病因,その病因とこれまで経験した症例から検討した診断のためのフローチャート,および,現存の治療法と私たちが試みた新しい治療法などを解説した. | |||||
抄録 | ||||||
内容記述タイプ | Abstract | |||||
内容記述 | TNF receptor-associated periodic syndrome (TRAPS) is an autosomal dominant inherited disease characterized by prolonged episodes of periodic fever and localized inflammation. The hypothetical pathogenesis of TRAPS is defective TNF receptor 1 (TNFRSF1A) shedding from cell membranes in response to a stimulus including TNFα. This mechanism has recently been shown to account for a minor population of TRAPS patients and other mechanisms are reported to explain the disease, such as resistance to apoptosis, TNFRSF1A internalization, or TNFRSF1A misfolding and aggregation, leading to NF-κB activation and apoptosis. Until now 15 TRAPS patients from 5 pedigree including 5 different mutations (C30R, C30Y, T61I, C70S, C70G) had been reported in Japan. There were many sporadic cases of TRAPS without TNFRSF1A mutation in our epidemiological study. In this issue, we described the clinical characterization, pathogenesis, diagnostic criteria, and treatment of TRAPS according to our case and literature. | |||||
書誌情報 |
日本臨床免疫学会会誌 en : Japanese Journal of Clinical Immunology 巻 30, 号 2, p. 90-100, 発行日 2007-04-28 |
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出版者 | ||||||
出版者 | 日本臨床免疫学会 | |||||
出版者別言語 | ||||||
The Japan Society for Clinical Immunology | ||||||
ISSN | ||||||
収録物識別子タイプ | ISSN | |||||
収録物識別子 | 09114300 | |||||
EISSN | ||||||
収録物識別子タイプ | ISSN | |||||
収録物識別子 | 13497413 | |||||
書誌レコードID | ||||||
収録物識別子タイプ | NCID | |||||
収録物識別子 | AN00357971 | |||||
DOI | ||||||
関連タイプ | isIdenticalTo | |||||
識別子タイプ | DOI | |||||
関連識別子 | 10.2177/jsci.30.90 | |||||
権利 | ||||||
権利情報 | Copyright (c) 2007 日本臨床免疫学会 | |||||
著者版フラグ | ||||||
出版タイプ | VoR | |||||
出版タイプResource | http://purl.org/coar/version/c_970fb48d4fbd8a85 | |||||
引用 | ||||||
内容記述タイプ | Other | |||||
内容記述 | 日本臨床免疫学会会誌, 30(2), pp.90-100; 2007 |