Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
1
pubmed:dateCreated
2009-1-5
pubmed:abstractText
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a malignant arrhythmia syndrome linked to mutations in the cardiac ryanodine receptor (RyR2) and calsequestrin (CASQ2). RyR2 and CASQ2 are parts of the multimolecular Ca(2+) release channel complex that is present on the sarcoplasmic reticulum (SR) to support myocyte Ca(2+) cycling and contractile activity. Whereas RyR2 operates as a Ca(2+) release channel, the SR Ca(2+) binding protein CASQ2 plays a dual role by serving as a SR Ca(2+) buffer and by regulating RyR2 function. Essential to stable Ca(2+) cycling, SR luminal Ca(2+)-dependent control of RyR2 activity by CASQ2 contributes to RyR2 deactivation and to the development of a temporary refractory state that occurs after each Ca(2+) release. Accumulating evidence suggests that the CPVT mutations act by reducing the extent and shortening the duration of Ca(2+) signaling refractoriness, thereby promoting untimely SR Ca(2+) release and arrhythmogenic delayed afterdepolarizations in cardiac myocytes. Similar mechanisms may apply to arrhythmias during various conditions, including heart failure and ischemic heart disease, associated with acquired defects in components of the Ca(2+) release channel complex.
pubmed:grant
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Jan
pubmed:issn
1556-3871
pubmed:author
pubmed:issnType
Electronic
pubmed:volume
6
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
123-9
pubmed:dateRevised
2009-10-27
pubmed:meshHeading
pubmed:year
2009
pubmed:articleTitle
Molecular basis of catecholaminergic polymorphic ventricular tachycardia.
pubmed:affiliation
Department of Physiology and Cell Biology, The Ohio State University, Columbus, Ohio 43210, USA. sandor.gyorke@osumc.edu
pubmed:publicationType
Journal Article, Review, Research Support, N.I.H., Extramural