Cardiac calsequestrin: the new kid on the block in arrhythmias.
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare inherited disease characterized by physical or emotional stress-induced ventricular arrhythmias in the absence of any structural heart disease or QT prolongation. Thus far, mutations in genes encoding the sarcoplasmic reticulum Ca(2+) release channel (RYR2) and the sarcoplasmic reticulum Ca(2+) binding protein cardiac calsequestrin (CASQ2) have been identified in CPVT patients. Here, we review the role of cardiac calsequestrin in health and disease, with a particular focus on how calsequestrin deficiency can cause arrhythmia susceptibility. Clinical implications and a promising new drug therapy for CPVT are discussed.
Chopra
Nagesh
N
Department of Medicine, Division of Cardiology, Vanderbilt University School of Medicine, Nashville, Tennessee 37232-0575, USA.
Knollmann
Björn C
BC
D000818:Animals; D001145:Arrhythmias, Cardiac / Q000503:physiopathology*; D002155:Calsequestrin / Q000378:metabolism*; D006329:Heart Conduction System / Q000503:physiopathology*; D006339:Heart Rate; D006801:Humans; D008955:Models, Cardiovascular; D009200:Myocardial Contraction
D002155:Calsequestrin