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 |
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| Section | 一般 |
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| Review Section |
Basic Section 53020:Cardiology-related
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| Research Institution | Hokkaido University |
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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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| Project Status |
Completed (Fiscal Year 2021)
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| Budget Amount *help |
¥4,420,000 (Direct Cost: ¥3,400,000、Indirect Cost: ¥1,020,000)
Fiscal Year 2021: ¥1,040,000 (Direct Cost: ¥800,000、Indirect Cost: ¥240,000)
Fiscal Year 2020: ¥910,000 (Direct Cost: ¥700,000、Indirect Cost: ¥210,000)
Fiscal Year 2019: ¥2,470,000 (Direct Cost: ¥1,900,000、Indirect Cost: ¥570,000)
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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 / 病理診断法 / 呼吸鎖酵素活性 / 免疫組織病理 / 呼吸鎖酵素複合体 |
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| Outline of Research at the Start |
ミトコンドリア心筋症の病理学的診断はこれまで確立されていない。本研究ではミトコンドリア病モデルマウスを用いたミトコンドリア心筋症の心筋超微形態を定量的に評価し、呼吸鎖酵素複合体に対する抗体を用いた免疫染色法を併用することにより病理的診断が可能かどうかを検証する。ヒトにおいても心筋生検で採取した組織を用いて電顕、免疫染色を行うことでミトコンドリア心筋症の診断を確立する。
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| 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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| 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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Report
(4 results)
Research Products
(15 results)
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[Journal Article] Long-term prognosis and genetic background of cardiomyopathy in 223 pediatric mitochondrial disease patients.2021
Author(s)
Imai-Okazaki A, Matsunaga A, Yatsuka Y, Nitta KR, Kishita Y, Sugiura A, Sugiyama Y, Fushimi T, Shimura M, Ichimoto K, Tajika M, Tominaga M, Ebihara T, Matsuhashi T, Tsuruoka T, Kohda M, Hirata T, Harashima H, Nojiri S, Takeda A, Nakaya A, Kogaki S, Sakata Y, Ohtake A, Murayama K, Okazaki Y.
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Journal Title
International Journal of Cardiology
Volume: 341
Pages: 48-55
DOI
Related Report
Peer Reviewed / Open Access
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[Journal Article] Advanced pathological study for definite diagnosis of mitochondrial cardiomyopathy2020
Author(s)
Takeda A, Murayama K, Okazaki Y, Imai-Okazaki A, Ohtake A, Takakuwa E, Yamazawa H, Izumi G, Abe J, Nagai A, Taniguchi K, Sasaki D, Tsujioka T, John M Basgen
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Journal Title
Journal of Clinical Pathology
Volume: -
Issue: 6
Pages: 365-371
DOI
Related Report
Peer Reviewed / Int'l Joint Research
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[Journal Article] Cardiomyopathy in children with mitochondrial disease: Prognosis and genetic background.2019
Author(s)
Imai-Okazaki A, Kishita Y, Kohda M, Mizuno Y, Fushimi T, Matsunaga A, Yatsuka Y, Hirata T, Harashima H, Takeda A, Nakaya A, Sakata Y, Kogaki S, Ohtake A, Murayama K, Okazaki Y.
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Journal Title
Int J Cardiol
Volume: 279
Pages: 115-121
DOI
Related Report
Peer Reviewed / Open Access
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