Sodium channels as macromolecular complexes: implications for inherited arrhythmia syndromes.

Sudden cardiac arrest due to a single sodium channel mutation producing a mixed phenotype of Brugada and Long QT3 syndromes

Inherited arrhythmia syndromes are a known, albeit rare, cause of sudden cardiac arrest which may present with characteristic electrocardiogram changes in patients with structurally normal heart. There are a variety of distinct arrhythmogenic syndromes that arise from mutations in voltage gated sodium channels, resulting in either gain or loss of function. We describe a patient with a primary i...

Inherited Cardiac Arrhythmia Syndromes

Lethal cardiac arrhythmias in individuals with structurally normal heart are often caused by variants in genes that encode cardiac ion channel and -subunits (102). Common inherited arrhythmia syndromes include the congenital long QT syndrome (LQTS), Brugada syndrome (BrS), short QT syndrome (SQT), and catecholaminergic polymorphic ventricular tachycardia (CPVT) (76). Moreover, inherited mutatio...

Leucine/isoleucine zipper coordination of ion channel macromolecular signaling complexes in the heart. Roles in inherited arrhythmias.

The sympathetic nervous system controls the force and rate of contraction of the heart. The rapid response to stress and exercise mediated by increased sympathetic nervous system (SNS) activity requires the coordinated regulation of several ion channels in response to activation of beta-adrenergic receptors. The microenvironment of target channels is mediated by the assembly of macromolecular s...

Cardiac sodium channel and inherited arrhythmia syndromes.

Effect of Sodium Valproate on Ouabain-Induced Arrhythmia in Isolated Guinea-Pig Atria

Sodium valproate (SV), an antiepileptic drug has several mechanism of action. It inhibits voltage sensitive Na+ channels and reduces intracellular Na accumulation. These actions are similar to that of both phenytoin and carbamazepine. We have investigated the direct cardiac action of SV and its effects on ouabain-induced arrhythmia in isolated guinea-pig atria. The guinea-pig atrium was dissect...