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Description

Familial hypertrophic cardiomyopathy (FHC) comprise about 270 mutations of sarcomeric (force generating) proteins. Surprisingly, about 15% of those with FHC are at a high risk of sudden cardiac death due to ventricular fibrillation, an electrical problem. This linkage is as surprising as working on the pistons of your car causing the headlights to flash. Mathematical modeling and large-scale simulations show how the force generating system might be linked to the electrical system through the calcium (Ca) control system. Sarcomeric protein mutations change the spatial topography of the molecules of the Ca control system. These topographic changes causes the Ca control system to become unstable and produce spontaneous Ca waves. These Ca waves, in turn, can cause electrical derangement and sudden cardiac death.