Article
Energy utilization of induced pluripotent stem cell-derived cardiomyocyte in Fabry disease.
International journal of cardiology - 1 Apr 2017
Chou Shih-Jie, Yu Wen-Chung, Chang Yuh-Lih, Chen Wen-Yeh, Chang Wei-Chao, Chien Yueh, Yen Jiin-Cherng, Liu Yung-Yang, Chen Shih-Jen, Wang Chien-Ying, Chen Yu-Han, Niu Dau-Ming, Lin Shing-Jong, Chen Jaw-Wen, Chiou Shih-Hwa, Leu Hsin-Bang
Abstract excerpt
BACKGROUND: Fabry disease (FD) is a lysosomal storage disease in which glycosphingolipids (GB3) accumulate in organs of the human body, leading to idiopathic hypertrophic cardiomyopathy and target organ damage. Its pathophysiology is still poorly understood. OBJECTIVES: We aimed to generate patient-specific induced pluripotent stem cells (iPSC) from FD patients presenting cardiomyopathy to determine whether the...
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