Desensitization of Myofilaments to Ca2+ as a Therapeutic Target for Hypertrophic Cardiomyopathy with Mutations in Thin Filament Proteins

摘要

BackgroundHypertrophic cardiomyopathy (HCM) is a common genetic disorder caused mainly by mutations in sarcomeric proteins and is characterized by maladaptive myocardial hypertrophy, diastolic heart failure, increased myofilament Ca²⁺ sensitivity and high susceptibility to sudden death. We tested the following hypothesis: correction of the increased myofilament sensitivity can delay or prevent the development of the HCM phenotype.