Establishment of diagnostic procedure and the new diagnostic criteria of mitochondrial cardiomyopathy based on an Imaging technology

2017 Fiscal Year Final Research Report

• Project/Area Number: 15K09679
• Research Category: Grant-in-Aid for Scientific Research (C)
• Allocation Type: Multi-year Fund
• Section: 一般
• Research Field: Pediatrics
• Research Institution: Hokkaido University
• Principal Investigator: Takeda Atsuhito 北海道大学, 大学病院, 助教 (00374413)
• Co-Investigator(Kenkyū-buntansha): 山田 勇磨 北海道大学, 薬学研究院, 准教授 (60451431)
• Research Collaborator: ABE JIRO 北海道大学, 北海道大学病院, 医員 (90802447)
• Project Period (FY): 2015-04-01 – 2018-03-31
• Keywords: ミトコンドリア / 電子顕微鏡 / 呼吸鎖酵素活性 / 心筋症

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.

Free Research Field

心筋症