TRPC3 Inhibitor Pyr3 Corrects the Disease-specific Phenotype of the Induced Pluripotent Stem Cell-derived Cardiomyocytes from the Patients with Infantile Hypertrophic Cardiomyopathy
Nihon Yakuri Gakkai nenkai yoshishu(2018)
摘要
Introduction: Hypertrophic cardiomyopathy (HCM) is a genetic disorder that is characterized by hypertrophy of the myocardium. Most of the patients with HCM show the symptoms and are diagnosed at adolescents or adults but some of the patients develop HCM at infancies. The etiologies of these infantile HCM include syndromic HCM and the prognosis of the infantile HCM, especially those diagnosed before 1-year-old, is worse compared with general HCM. Nevertheless, pathophysiology of the infantile HCM, including the similarity and difference between general HCM, remains largely unknown.
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