Endothelin‐1 Induces Myofibrillar Disarray and Contractile Vector Variability in Hypertrophic Cardiomyopathy–Induced Pluripotent Stem Cell–Derived Cardiomyocytes

• Published in: Journal of the American Heart Association Vol. 3; no. 6; pp. e001263 - n/a
• Main Authors: Tanaka, Atsushi, Yuasa, Shinsuke, Mearini, Giulia, Egashira, Toru, Seki, Tomohisa, Kodaira, Masaki, Kusumoto, Dai, Kuroda, Yusuke, Okata, Shinichiro, Suzuki, Tomoyuki, Inohara, Taku, Arimura, Takuro, Makino, Shinji, Kimura, Kensuke, Kimura, Akinori, Furukawa, Tetsushi, Carrier, Lucie, Node, Koichi, Fukuda, Keiichi
• Format: Journal Article
• Language: English
• Published: England Blackwell Publishing Ltd 11.11.2014
• Subjects: Animals; Biomechanical Phenomena; Cardiomegaly - genetics; Cardiomegaly - metabolism; Cardiomegaly - pathology; Cardiomegaly - physiopathology; Cardiomyopathy, Hypertrophic - metabolism; Cardiomyopathy, Hypertrophic - pathology; Cardiomyopathy, Hypertrophic - physiopathology; Carrier Proteins - genetics; Carrier Proteins - metabolism; Case-Control Studies; Cell Differentiation; Cells, Cultured; disease modeling; Dose-Response Relationship, Drug; Endothelin-1 - pharmacology; ET‐1; Gene-Environment Interaction; Genotype; HCM; Humans; Induced Pluripotent Stem Cells - drug effects; Induced Pluripotent Stem Cells - metabolism; Induced Pluripotent Stem Cells - pathology; iPS cells; Mice, Inbred NOD; Mice, SCID; Mice, Transgenic; MYBPC3; Myocardial Contraction - drug effects; Myocytes, Cardiac - drug effects; Myocytes, Cardiac - metabolism; Myocytes, Cardiac - pathology; Myofibrils - drug effects; Myofibrils - metabolism; Myofibrils - pathology; Original Research; Phenotype; Risk Factors; Signal Transduction - drug effects; Time Factors; Transfection; Ventricular Dysfunction - genetics; Ventricular Dysfunction - metabolism; Ventricular Dysfunction - pathology; Ventricular Dysfunction - physiopathology; MYBPC3; HCM; ET‐1; disease modeling; iPS cells

Background Despite the accumulating genetic and molecular investigations into hypertrophic cardiomyopathy (HCM), it remains unclear how this condition develops and worsens pathologically and clinically in terms of the genetic–environmental interactions. Establishing a human disease model for HCM wou...