2021 Fiscal Year Final Research Report
Establishment of the new diagnostic method in mitochondrial cardiomyopathy
Project/Area Number |
19K08529
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Research Category |
Grant-in-Aid for Scientific Research (C)
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Allocation Type | Multi-year Fund |
Section | 一般 |
Review Section |
Basic Section 53020:Cardiology-related
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Research Institution | Hokkaido University |
Principal Investigator |
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Co-Investigator(Kenkyū-buntansha) |
永井 礼子 北海道大学, 大学病院, 医員 (10622160)
山田 勇磨 北海道大学, 薬学研究院, 准教授 (60451431)
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Project Period (FY) |
2019-04-01 – 2022-03-31
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Keywords | ミトコンドリア心筋症 / 電子顕微鏡 / Stereology / 免疫染色法 |
Outline of Final Research Achievements |
Mitochondrial cardiomyopathy (MCM), one of the secondary cardiomyopathies, is diagnosed definitively by pathology, tissue biochemistry, and genetic testing, but there are no clear criteria for myocardial pathology. Therefore, we measured mitochondrial volume density in electron microscopy images and performed immunohistochemistry using respiratory chain enzyme antibodies in MCMs diagnosed definitively by clinical symptoms, genetic testing, and tissue biochemistry, and compared them with other cardiomyopathies other than MCM. Mitochondrial volume density was significantly higher in the MCM group, and immunohistochemistry showed decreased staining for Complex I or IV in the MCM group. This study allowed the pathological diagnosis of MCM using electron microscopy and immunohistochemistry, which is very useful for the pathological criteria of MCM.
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Free Research Field |
小児科
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Academic Significance and Societal Importance of the Research Achievements |
ミトコンドリア心筋症の確定診断法について病理学的に確立し、二次性心筋症の鑑別診断に大きく貢献した。特に呼吸鎖酵素抗体による免疫染色法による診断法の開発により、ホルマリン固定のみの検体でも病理診断が可能であることを示した。ミトコンドリア心筋症は乳児発症例の生命予後が不良であり乳幼児突然死症候群の一因としても考えられている。したがって、本診断法はチャイルド・デス。レビューの死因究明にも応用できる。
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