2021 Fiscal Year Final Research Report
Elucidation of the pathogenesis of inclusion body myositis by focusing on the abnormal aggregation protein clearance system
| Project/Area Number |
20K21563
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| Research Category |
Grant-in-Aid for Challenging Research (Exploratory)
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| Allocation Type | Multi-year Fund |
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| Review Section |
Medium-sized Section 52:General internal medicine and related fields
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| Research Institution | Tohoku University |
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Principal Investigator |
AOKI Masashi 東北大学, 医学系研究科, 教授 (70302148)
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| Co-Investigator(Kenkyū-buntansha) |
割田 仁 東北大学, 大学病院, 助教 (30400245)
鈴木 直輝 東北大学, 大学病院, 助教 (70451599)
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| Project Period (FY) |
2020-07-30 – 2022-03-31
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| Keywords | 封入体筋炎 |
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| Outline of Final Research Achievements |
Inclusion body myositis (sIBM) is a chronic, progressive and refractory muscle disease seen in middle-aged and older people. sIBM patients' skeletal muscles show abnormal aggregates such as amyloid-beta, TDP-43 and FUS, suggesting a degenerative disease in the muscle. Using an originally developed human skeletal muscle electrical pulse culture system, we found cytoplasmic deposition of TDP-43, one of the aggregation proteins found in ALS. The results were reported in SciRep journal. Myoblasts were established from three new cases of sIBM muscle biopsies. B-cell follicle in sIBM muscle tissue were also observed in a case. It was summarised and reported in NeuromusDisord journal.
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| Free Research Field |
臨床神経学
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| Academic Significance and Societal Importance of the Research Achievements |
封入体筋炎は治療法が無く最終的に著明な筋萎縮を来して寝たきりになる難病であり、日本人でも社会の高齢化に伴い、患者数が増加しており、中高年で最も頻度の高い炎症性筋疾患となっている。その病態解明・治療開発は高齢化に伴うサルコペニアやフレイル、廃用性筋萎縮・筋ジストロフィーなどへの治療開発に寄与しうる。本研究により異常蛋白が蓄積するという変性の観点から、封入体筋炎の病態の一旦が明らかになった。
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