Clinical features and molecular pathology of anti-Lrp4 antibody-positive motor neuron disorders
| Project/Area Number |
15K08636
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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 |
Laboratory medicine
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| Research Institution | Nagasaki Kawatana Medical Center |
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Principal Investigator |
Higuchi Osamu 独立行政法人国立病院機構長崎川棚医療センター(臨床研究部), その他の研究科, 研究員(移行) (50361720)
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| Co-Investigator(Kenkyū-buntansha) |
瀬筒 秀樹 国立研究開発法人農業・食品産業技術総合研究機構, 生物機能利用研究部門, ユニット長 (70342805)
中根 俊成 熊本大学, 大学院生命科学研究部(医), 寄附講座教員 (70398022)
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| Research Collaborator |
瀬筒 秀樹 (70342805)
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| Project Period (FY) |
2015-04-01 – 2018-03-31
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| Project Status |
Completed (Fiscal Year 2017)
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| Budget Amount *help |
¥4,940,000 (Direct Cost: ¥3,800,000、Indirect Cost: ¥1,140,000)
Fiscal Year 2017: ¥780,000 (Direct Cost: ¥600,000、Indirect Cost: ¥180,000)
Fiscal Year 2016: ¥2,210,000 (Direct Cost: ¥1,700,000、Indirect Cost: ¥510,000)
Fiscal Year 2015: ¥1,950,000 (Direct Cost: ¥1,500,000、Indirect Cost: ¥450,000)
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| Keywords | autoantobody / ALS / neuroimmunology / surrogatemarker / epitope / agrin / LRP4 / MG / エピトープ / 抗体 / 抗原 / 重症筋無力症 / 自己抗体 / 運動ニューロン / 神経変性疾患 |
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| Outline of Final Research Achievements |
As a result of searching for LRP4 antibody in patient serum of amyotrophic lateral sclerosis (ALS) which is one of neurodegenerative diseases, LRP4 antibody was positive in 40-50% of cases. The inhibitory effect on agrin-induced AChR clustering activity in mouse myotube cells was examined. As a result, the serum of patients with LRP4 antibody positive myasthenia gravis had its inhibitory effect, but in sera of LRP4 antibody-positive ALS patients it I did not find it. It is speculated that the results of this study reflect the difference in the epitope of the disease-specific LRP4 antibody.
The LRP4 antibody found in the blood of ALS patients may be expected to play a role as a surrogate marker capable of tracking ALS onset and therapeutic responsiveness in the future.
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Report
(4 results)
Research Products
(18 results)
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[Journal Article] Myasthenic symptoms in anti-low-density lipoprotein receptor-related protein 4 antibody-seropositive amyotrophic lateral sclerosis: two case reports.2016
Author(s)
Takahashi H, Noto YI, Makita N, Kushimura-Okada Y, Ishii R, Tanaka A, Ohara T, Nakane S, Higuchi O, Nakagawa M, Mizuno T
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Journal Title
BMC neurology
Volume: 16
Issue: 1
Pages: 229-229
DOI
Related Report
Peer Reviewed / Open Access / Int'l Joint Research
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[Presentation] repetitive CMAPを認め,神経筋接合部の微細構造を解析し得た抗LRP4抗体陽性重症筋無力症の一例2017
Author(s)
光武明彦, 平賢一郎, 荒川晶, 佐藤達哉, 勝又淳子, 関大成, 前川理沙, 日出山拓人, 石浦浩之, 内尾直裕, 清水潤, 樋口理, 松尾秀徳, 椎尾康
Organizer
日本神経免疫学会
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