Article
Pathogenesis of Hypertrophic Cardiomyopathy is Mutation Rather Than Disease Specific: A Comparison of the Cardiac Troponin T E163R and R92Q Mouse Models.
Journal of the American Heart Association - 22 Jul 2017
Ferrantini Cecilia, Coppini Raffaele, Pioner Josè Manuel, Gentile Francesca, Tosi Benedetta, Mazzoni Luca, Scellini Beatrice, Piroddi Nicoletta, Laurino Annunziatina, Santini Lorenzo, Spinelli Valentina, Sacconi Leonardo, De Tombe Pieter, Moore Rachel, Tardiff Jil, Mugelli Alessandro, Olivotto Iacopo, Cerbai Elisabetta, Tesi Chiara, Poggesi Corrado
Abstract excerpt
BACKGROUND: In cardiomyocytes from patients with hypertrophic cardiomyopathy, mechanical dysfunction and arrhythmogenicity are caused by mutation-driven changes in myofilament function combined with excitation-contraction (E-C) coupling abnormalities related to adverse remodeling. Whether myofilament or E-C coupling alterations are more relevant in disease development is unknown. Here, we aim to investigate...
Topics
- Animals
- Calcium Signaling
- Calcium-Calmodulin-Dependent Protein Kinases
- Cardiomyopathy, Hypertrophic
- Disease Models, Animal
- Excitation Contraction Coupling
- Fibrosis