Pacing Dynamics Determines the Arrhythmogenic Mechanism of the CPVT2-Causing CASQ2^G112+5X Mutation in a Guinea Pig Ventricular Myocyte Computational Model.

ABSTRACT: Calsequestrin Type 2 (CASQ2) is a high-capacity, low-affinity, Ca²⁺-binding protein expressed in the sarcoplasmic reticulum (SR) of the cardiac myocyte. Mutations in CASQ2 have been linked to the arrhythmia catecholaminergic polymorphic ventricular tachycardia (CPVT2) that occurs with acute emotional stress or exercise can result in sudden cardiac death (SCD). CASQ2^G112+5X is a 16 bp (339-354) deletion CASQ2 mutation that prevents the protein expression due to premature stop codon. Understanding the subcellular mechanisms of CPVT2 is experimentally challenging because the occurrence of arrhythmia is rare. To obtain an insight into the characteristics of this rare disease, simulation studies using a local control stochastic computational model of the Guinea pig ventric

SUBMITTER: Paudel R 

PROVIDER: S-EPMC9858930 | biostudies-literature | 2022 Dec

REPOSITORIES: biostudies-literature