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...

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Publicado en:Journal of Cardiovascular Electrophysiology Vol. 20; no. 10; pp. 1179 - 1186
Autores principales: Chopra N, Knollmann BC
Formato: tables/charts tracings Journal Article
Publicado: Wiley-Blackwell Oct2009
Acceso en línea:Ver este registro en EBSCOhost
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        atl: Cardiac calsequestrin: the new kid on the block in arrhythmias.
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          Chopra N
          Knollmann BC
      sug:
        subj:
          Adrenergic beta-Antagonists Administration and Dosage
          Arrhythmia Risk Factors
          Catecholamines Adverse Effects
          Genetics
          Protein Deficiency Familial and Genetic
          Arrhythmia Physiopathology
          Exercise Adverse Effects
          Microscopy, Electron
          Mutation
          Stress Complications
      ab: 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.
      pubtype: Academic Journal
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        Journal Article
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    language: English
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