Complex Arrhythmia Syndrome in a Knock-In Mouse Model Carrier of the N98S Calm1 Mutation.
Conclusions: Heterozygosity for the Calm1N98S mutation is causative of an arrhythmia syndrome characterized by sinus bradycardia, QRS widening, adrenergically mediated QTc interval prolongation and bidirectional ventricular tachycardia. β-adrenergically induced ICa.L dysregulation contributes to the long QT phenotype. Pause-dependent early afterdepolarizations and tachycardia-induced delayed afterdepolarizations originating in the His-Purkinje network and ventricular myocytes, respectively, constitute potential sources of arrhythmia in Calm1N98S/+ hearts.
PMID: 32929985 [PubMed - as supplied by publisher]
Source: Circulation - Category: Cardiology Authors: Tsai WC, Guo S, Olaopa MA, Field LJ, Yang J, Shen C, Chang CP, Chen PS, Rubart M Tags: Circulation Source Type: research