BMB Rep. 2015; 48(10): 541-548  
An experimental approach to study the function of mitochondria in cardiomyopathy
Youn Wook Chung1 & Seok-Min Kang1,2,3,*
1Yonsei Cardiovascular Research Institute, Yonsei University College of Medicine, Seoul 03722, 2Cardiology Division, Severance Cardiovascular Hospital, Seoul 03722, 3Severance Integrative Research Institute for Cerebral and Cardiovascular Diseases (SIRIC), Yonsei University Health System, Seoul 03722, Korea
Correspondence to: Tel: +82-2-2228-1042; Fax: +82-2-363-8676; E-mail: smkang@yuhs.ac
Received: July 20, 2015; Published online: October 31, 2015.
© Korean Society for Biochemistry and Molecular Biology. All rights reserved.

Abstract
Cardiomyopathy is an inherited or acquired disease of the myocardium, which can result in severe ventricular dysfunction. Mitochondrial dysfunction is involved in the pathological process of cardiomyopathy. Many dysfunctions in cardiac mitochondria are consequences of mutations in nuclear or mitochondrial DNA followed by alterations in transcriptional regulation, mitochondrial protein function, and mitochondrial dynamics and energetics, presenting with associated multisystem mitochondrial disorders. To ensure correct diagnosis and optimal management of mitochondrial dysfunction in cardiomyopathy caused by multiple pathogenesis, multidisciplinary approaches are required, and to integrate between clinical and basic sciences, ideal translational models are needed. In this review, we will focus on experimental models to provide insights into basic mitochondrial physiology and detailed underlying mechanisms of cardiomyopathy and current mitochondria-targeted therapies for cardiomyopathy.
Keywords: Cardiomyopathy, Heart, Mitochondria


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