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Titolo:
Involvement of the cardiac ryanodine receptor/calcium release channel in catecholaminergic polymorphic ventricular tachycardia
Autore:
Marks, AR; Priori, S; Memmi, M; Kontula, K; Laitinen, PJ;
Indirizzi:
Columbia Univ Coll Phys & Surg, Ctr Mol Cardiol, Dept Pharmacol, New York,NY 10032 USA Columbia Univ Coll Phys & Surg New York NY USA 10032 ew York,NY 10032 USA Columbia Univ Coll Phys & Surg, Ctr Mol Cardiol, Dept Med, New York, NY 10032 USA Columbia Univ Coll Phys & Surg New York NY USA 10032 w York, NY 10032 USA Univ Pavia, IRCCS, Mol Cardiol Lab, I-27100 Pavia, Italy Univ Pavia Pavia Italy I-27100 CS, Mol Cardiol Lab, I-27100 Pavia, Italy Univ Helsinki, Dept Med, Helsinki, Finland Univ Helsinki Helsinki Finland iv Helsinki, Dept Med, Helsinki, Finland
Titolo Testata:
JOURNAL OF CELLULAR PHYSIOLOGY
fascicolo: 1, volume: 190, anno: 2002,
pagine: 1 - 6
SICI:
0021-9541(200201)190:1<1:IOTCRR>2.0.ZU;2-N
Fonte:
ISI
Lingua:
ENG
Soggetto:
CENTRAL CORE DISEASE; MALIGNANT HYPERTHERMIA; CALCIUM-RELEASE; INTRACELLULAR CALCIUM; CA2+ RELEASE; DEPENDENT PHOSPHORYLATION; SARCOPLASMIC-RETICULUM; CHROMOSOME 1Q42-Q43; BINDING-PROTEIN; MUSCLE;
Tipo documento:
Review
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
65
Recensione:
Indirizzi per estratti:
Indirizzo: Marks, AR Columbia Univ Coll Phys & Surg, Ctr Mol Cardiol, Dept Pharmacol,Box 65,Rm9-401,630 W 168th St, New York, NY 10032 USA Columbia Univ Coll Phys & Surg Box 65,Rm 9-401,630 W 168th St New York NY USA 10032
Citazione:
A.R. Marks et al., "Involvement of the cardiac ryanodine receptor/calcium release channel in catecholaminergic polymorphic ventricular tachycardia", J CELL PHYS, 190(1), 2002, pp. 1-6

Abstract

The cardiac ryanodine receptor (RyR2), the major calcium release channel on the sarcoplasmic reticulum (SR) in cardiomyocytes, has recently been shown to be involved in at least two forms of sudden cardiac death (SCD): (1) Catecholaminergic polymorphic ventricular tachycardia (CPVT) or familial polymorphic VT (FPVT); and (2) Arrhythmogenic right ventricular dysplasia type2 (ARVD2). Eleven RyR2 missense mutations have been linked to these diseases. All eleven RyR2 mutations cluster into 3 regions of RyR2 that are homologous to the three malignant hyperthermia (MH)/central core disease (CCD) mutation regions of the skeletal muscle ryanodine receptor/calcium release channel RyR1. MH/CCD RyR1 mutations have been shown to alter calcium-inducedcalcium release. Sympathetic nervous system stimulation leads to phosphorylation of RyR2 by protein kinase A (PKA). PKA phosphorylation of RyR2 activates the channel. In conditions associated with high rates of SCD such as heart failure RyR2 is PICA hyperphosphorylated resulting in "leaky" channels. SR calcium leak during diastole can generate "delayed after depolarizations" that can trigger fatal cardiac arrhythmias (e.g., VT). We propose that RyR2 mutations linked to genetic forms of catecholaminergic-induced SCE) may alter the regulation of the channel resulting in increased SR calcium leak during sympathetic stimulation. (C) 2002 Wiley-Liss, Inc.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 05/04/20 alle ore 19:40:00