Deregulated Ca ²⁺ cycling underlies the development of arrhythmia and heart disease due to mutant obscurin

Abstract

deregulation and spontaneous arrhythmia, whereas in the presence of chronic, pathological stress, it leads to cardiac remodeling and dilation. We postulate that enhanced binding between mutant obscurins and phospholamban leads to SERCA2 disinhibition, which may underlie the observed pathological alterations.