Clinical pictures and neuromuscular junction pathomechanism in novel LDL- recepotr related protein 4 antibody-positive myasthenia gravis
| Project/Area Number |
23591248
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| Research Category |
Grant-in-Aid for Scientific Research (C)
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| Allocation Type | Multi-year Fund |
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| Section | 一般 |
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| Research Field |
Neurology
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| Research Institution | Nagasaki Institute of Applied Science (2013)
Nagasaki University (2011-2012) |
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Principal Investigator |
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| Co-Investigator(Kenkyū-buntansha) |
FUKUDA Taku 長崎大学, 大学病院, 研究員 (50437894)
YOSHIMURA Toshiro 長崎大学, 大学院医歯薬学総合研究科, 教授 (80182822)
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| Project Period (FY) |
2011 – 2013
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| Project Status |
Completed (Fiscal Year 2013)
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| Budget Amount *help |
¥5,460,000 (Direct Cost: ¥4,200,000、Indirect Cost: ¥1,260,000)
Fiscal Year 2013: ¥1,040,000 (Direct Cost: ¥800,000、Indirect Cost: ¥240,000)
Fiscal Year 2012: ¥1,040,000 (Direct Cost: ¥800,000、Indirect Cost: ¥240,000)
Fiscal Year 2011: ¥3,380,000 (Direct Cost: ¥2,600,000、Indirect Cost: ¥780,000)
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| Keywords | 重症筋無力症 / 自己抗体 / 神経筋接合部疾患 / 新規自己抗体 / Lrp4 / LDL受容体関連蛋白質４ / アセチルコリン受容体 / 筋特異的受容体型チロシンキナーゼ |
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| Research Abstract |
Myasthenia gravis (MG) is caused by the failure of neuromuscular transmission mediated by pathogenic autoantibodies (Abs) against acetylcholine receptor (AChR) and muscle-specific receptor tyrosine kinase (MuSK). The seropositivity rates for routine AChR binding Ab and MuSK Ab in MG are 80-85% and 5-10% for MG patients in Japan, respectively. In 2011, autoantibodies against low-density lipoprotein receptor-related protein 4 (Lrp4) were identified in Japanese MG patients. We developed a simple technique termed Gaussia luciferase immunoprecipitation for detecting antibodies to Lrp4. As a result, nine generalized MG patients from 300 lacking AChR Ab are positive for Lrp4 antibodies. These antibodies inhibit binding of Lrp4 to its ligand and are predominantly of the IgG1 subclass. Further studies including neuromuscular junction biopsy are needed to clarify the pathomechanism of Lrp4 ab positive MG. These results indicate that Lrp4 is a third pathogenic autoantigen in patients with MG.
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Report
(4 results)
Research Products
(67 results)
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[Journal Article] 放射性免疫測定法による筋特異的受容体型チロシンキナーゼ抗体測定キットの基礎的・臨床的検討2013
Author(s)
本村政勝,中田るか,白石裕一,枡田智子,福田卓,徳田昌紘,中嶋秀樹,宮崎禎一郎,向野晃弘,前田泰宏,太田理絵,吉村俊祐,吉村俊朗,川上純,佐藤聡,辻畑光宏, 菊池強
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Journal Title
医学と薬学
Volume: 70(2)
Pages: 421-428
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[Journal Article] Childhood-onset anti-MuSK antibody positive myasthenia gravis demonstrates a distinct clinical course2012
Author(s)
Takahashi Y,Sugiyama M, Ueda Y, Itoh T, Yagyu K, Shiraishi H, Ukeba-Terashita Y, Nakanishi M, Nagashima T, Imai T, Motomura M, Saitoh S
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Journal Title
Brain Dev
Volume: 34(9)
Pages: 784-786
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Peer Reviewed
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[Journal Article] Characteristics of myasthenia gravis according to onset-age : Japanese nationwide survey2011
Author(s)
Murai H, Yamashita N, Watanabe M, Nomura Y, Motomura M, Yoshikawa H, Nakamura Y, Kawaguchi N, Onodera H, Araga S, Isobe N, Nagai M, Kira J
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Journal Title
J Neurol Sci
Volume: 305(1-2)
Pages: 97-102
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Peer Reviewed
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[Presentation] 受動免疫性重症筋無力症ラットの神経筋接合部分子病態2013
Author(s)
本村政勝, 吉村俊祐, 吉村俊郎, 成田智子, 中田るか, 向野晃弘, 宮崎禎一郎, 中嶋秀樹, 福田 卓, 川上 純, 江口円俊, 樋口 理, 辻畑光宏
Organizer
第54回 日本神経学会学術大会 東京国際フォーラム
Place of Presentation
東京国際フォーラム(東京都千代田区)
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[Presentation] 重症筋無力症におけるAChRMRI抗体価とその臨床像2012
Author(s)
枡田智子,本村政勝,槍澤公明,長根百合子,中田るか,徳田昌紘,中嶋秀樹,宮崎禎一郎,吉村俊祐,福田卓,吉村俊朗,辻畑光宏,川上純
Organizer
第53回日本神経学会学術大会
Place of Presentation
東京国際フォーラム(ポスター発表)
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[Presentation] Lambert-Eaton筋無力症候群における嚥下機能障害に対する検討2012
Author(s)
湯淺隆行,本村政勝,枡田智子,中田るか,佐藤聡,辻畑光宏,川上純,吉村俊朗,松下新子,久松徳子,小山善哉,石飛進吾
Organizer
第53回日本神経学会学術大会
Place of Presentation
東京国際フォーラム(ポスター発表)
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[Presentation] コリンエステラーゼ阻害剤投与マウスとCollargenQ欠損マウスにおける運動終板微細構造の比較2011
Author(s)
吉村俊朗,伊藤美佳子,片岡英樹,福留隆泰,Krejci Eric,岡田尚己,武田伸一,本村政勝,辻野彰,吉村俊祐,枡田智子,中田るか,徳田昌紘,福田卓,大野欽司
Organizer
第52回日本神経学会学術大会
Place of Presentation
名古屋国際会議場(ポスター)
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