Article
Impaired Relaxation in Induced Pluripotent Stem Cell-Derived Cardiomyocytes with Pathogenic TNNI3 Mutation of Pediatric Restrictive Cardiomyopathy.
Journal of the American Heart Association - 19 Mar 2024
Wang Renjie, Hasegawa Moyu, Suginobe Hidehiro, Yoshihara Chika, Ishii Yoichiro, Ueyama Atsuko, Ueda Kazutoshi, Hashimoto Kazuhisa, Hirose Masaki, Ishii Ryo, Narita Jun, Watanabe Takuji, Kawamura Takuji, Taira Masaki, Ueno Takayoshi, Miyagawa Shigeru, Ishida Hidekazu
Abstract excerpt
BACKGROUND: Restrictive cardiomyopathy (RCM) is characterized by impaired diastolic function with preserved ventricular contraction. Several pathogenic variants in sarcomere genes, including TNNI3, are reported to cause Ca2+ hypersensitivity in cardiomyocytes in overexpression models; however, the pathophysiology of induced pluripotent stem cell (iPSC)-derived cardiomyocytes specific to a patient with RCM remains...
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