Establishment of diagnostic procedure and the new diagnostic criteria of mitochondrial cardiomyopathy based on an Imaging technology
Project/Area Number |
15K09679
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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 |
Research Field |
Pediatrics
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Research Institution | Hokkaido University |
Principal Investigator |
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Co-Investigator(Kenkyū-buntansha) |
山田 勇磨 北海道大学, 薬学研究院, 准教授 (60451431)
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Research Collaborator |
ABE JIRO 北海道大学, 北海道大学病院, 医員 (90802447)
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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 |
¥4,810,000 (Direct Cost: ¥3,700,000、Indirect Cost: ¥1,110,000)
Fiscal Year 2017: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000)
Fiscal Year 2016: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
Fiscal Year 2015: ¥1,820,000 (Direct Cost: ¥1,400,000、Indirect Cost: ¥420,000)
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Keywords | ミトコンドリア / 電子顕微鏡 / 呼吸鎖酵素活性 / 心筋症 / ミトコンドリア心筋症 / ステレオロジー / イメージング |
Outline of Final Research Achievements |
Mitochondrial disease, which is a designated intractable disease, has not yet been established as a treatment method, and it is urgent to establish a diagnostic method leading to early detection. We have aimed to establish mitochondrial cardiomyopathy diagnostic criteria including mitochondrial cardiomyopathy model and its evaluation system. In the basic experiments, the results were confirmed in the preparation and evaluation system of doxorubicin mitochondrial cardiomyopathy-induced model animals and the rescue experiment using mitochondrial DDS. Clinically, as diagnostic criteria for mitochondrial cardiomyopathy, we identified three points of (1) myocardial pathology tissue: mitochondrial abnormality in electron microscopic image, (2) tissue biochemistry: myocardial tissue respiratory chain enzyme activity reduction, and (3) gene mutation as necessary elements.
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Report
(4 results)
Research Products
(70 results)
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[Journal Article] Barth syndrome: Different approaches to diagnosis2018
Author(s)
Imai-Okazaki A, Kishita Y, Kohda M, Yatsuka Y, Hirata T, Mizuno Y, Harashima H, Hirono K, Ichida F, Noguchi A, Yoshida M, Tokorodani C, Nishiuchi R, Takeda A, Nakaya A, Sakata Y, Murayama K, Ohtake A, & Okazaki Y
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Journal Title
Journal of Pediatrics
Volume: 193
Pages: 256-260
DOI
Related Report
Peer Reviewed / Open Access
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[Journal Article] A novel mutation in TAZ causes mitochondrial respiratory chain disorder without cardiomyopathy2016
Author(s)
Borna NN, Kishita Y, Ishikawa K, Nakada K, Hayashi JI, Tokuzawa Y, Kohda M, Nyuzuki H, Yamashita-Sugahara Y, Nasu T, Takeda A, Murayama K, Ohtake A, Okazaki Y
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Journal Title
J Hum Genet
Volume: 62
Issue: 5
Pages: 539-547
DOI
NAID
Related Report
Peer Reviewed / Int'l Joint Research
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[Journal Article] Rapidly progressive infantile cardiomyopathy with mitochondrial respiratory chain complex V deficiency due to loss of ATPase 6 and 8 protein.2016
Author(s)
Imai A, Fujita S, Kishita Y, Kohda M, Tokuzawa Y, Hirata T, Mizuno Y, Harashima H, Nakaya A, Sakata Y, Takeda A, Mori M, Murayama K, Ohtake A, Okazaki Y
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Journal Title
Int J Cardiol.
Volume: 15
Pages: 203-205
DOI
Related Report
Peer Reviewed
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[Presentation] A novel mutation in TAZ causes mitochondrial respiratory chain disorder without cardiomyopathy2016
Author(s)
Nurun Nahar Borna, Yoshihito Kishita, Kaori Ishikawa, Kazuto Nakada, Jun-Ichi Hayashi, Yoshimi Tokuzawa, Masakazu Kohda, Hiromi Nyuzuki, Yzumi Yamashita-Sugahara1,Takashi Nasu, Atsuhito Takeda, Kei Murayama, Akira Ohtake, Yasushi Okazaki
Organizer
Conference of Asian Society for Mitochondrial Research and Medicine
Place of Presentation
TKPガーデンシティ品川 (東京都品川区)
Related Report
Int'l Joint Research
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