Document Detail


Role of Calmodulin Kinase in Catecholaminergic Polymorphic Ventricular Tachycardia.
MedLine Citation:
PMID:  21397047     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited disease causing arrhythmias and sudden death in a structurally normal heart. Ventricular and supraventricular arrhythmias are reproducibly triggered by exercise or acute emotion (i.e. sudden adrenergic activation). Two CPVT variants are known: autosomal dominant caused by mutations in the cardiac ryanodine receptor (RyR2) and autosomal recessive due to calsequestrin mutations (CASQ2). Both RyR2 and CASQ2 have a relevant role in the control of intracellular Ca(2+) fluxes. Thus, CPVT pathogenesis is intrinsically bound to catecholamines and Ca(2+) handling. Cardiac Ca(2+)/calmodulin-dependent protein kinase (CaMKII) is an intracellular mediator of the adrenergic cascade and it specifically modulates Ca(2+) homeostasis. Several lines of evidence suggest that CaMKII not only has a key role in CPVT pathogenesis but is also a promising therapeutic target. Here we will review the available evidence on both these issues and the possible future developments in this area.
Authors:
Carlo Napolitano; Nian Liu; Silvia G Priori
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-3-9
Journal Detail:
Title:  Heart rhythm : the official journal of the Heart Rhythm Society     Volume:  -     ISSN:  1556-3871     ISO Abbreviation:  -     Publication Date:  2011 Mar 
Date Detail:
Created Date:  2011-3-14     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101200317     Medline TA:  Heart Rhythm     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
Copyright © 2011. Published by Elsevier Inc.
Affiliation:
Cardiovascular Genetics Program, The Leon H. Charney Division of Cardiology, New York University School of Medicine, New York, USA; Molecular Cardiology, IRCCS Fondazione Salvatore Maugeri , Pavia, Italy.
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