Key result
CPVT-associated CaM mutants promoted significantly higher spontaneous Ca wave and spark activity and greater RyR2 single-channel open probability compared with wild-type CaM, whereas LQTS-CaMs did not.
CPVT-associated calmodulin mutations, but not LQTS-associated mutations, promote arrhythmogenic calcium disturbances by enhancing RyR2 single-channel open probability and binding affinity.
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Distinguishes CPVT from LQTS mechanisms in calmodulin mutations; hypothesis-generating for targeted therapies pending human validation.
Hwang et al. (2014) studied Catecholaminergic polymorphic ventricular tachycardia (CPVT) or long QT syndrome (LQTS). Recombinant CaM mutants associated with CPVT (N54I and N98S) vs. Wild-type CaM and LQTS-associated CaM mutants was evaluated on Spontaneous Ca wave and spark activity, and RyR2 single-channel open probability. CPVT-associated CaM mutants promoted significantly higher spontaneous Ca wave and spark activity and greater RyR2 single-channel open probability compared with wild-type CaM, whereas LQTS-CaMs did not.
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