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 C...
| Publicado en: | Journal of Cardiovascular Electrophysiology Vol. 20; no. 10; pp. 1179 - 1186 |
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| Autores principales: | , |
| Formato: | tables/charts tracings Journal Article |
| Publicado: |
Wiley-Blackwell
Oct2009
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| Acceso en línea: | Ver este registro en EBSCOhost |
| Sumario: | 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 Ca2+ release channel (RYR2) and the sarcoplasmic reticulum Ca2+ 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. |
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