Clinical features and molecular pathology of anti-Lrp4 antibody-positive motor neuron disorders

2017 Fiscal Year Final Research Report

• Project/Area Number: 15K08636
• Research Category: Grant-in-Aid for Scientific Research (C)
• Allocation Type: Multi-year Fund
• Section: 一般
• Research Field: Laboratory medicine
• Research Institution: Nagasaki Kawatana Medical Center
• 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)
• 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)
• Research Collaborator: 瀬筒 秀樹 (70342805)
• Project Period (FY): 2015-04-01 – 2018-03-31
• Keywords: autoantobody / ALS / neuroimmunology / surrogatemarker / epitope

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.

Free Research Field

神経免疫学