Ryanodine receptors and ventricular arrhythmias: emerging trends in mutations, mechanisms and therapies

CH George, H Jundi, NL Thomas, DL Fry… - Journal of molecular and …, 2007 - Elsevier
It has been six years since the first reported link between mutations in the cardiac ryanodine
receptor Ca2+ release channel (RyR2) and catecholaminergic polymorphic ventricular …

Molecular, subcellular, and arrhythmogenic mechanisms in genetic RyR2 disease

ED Fowler, S Zissimopoulos - Biomolecules, 2022 - mdpi.com
The ryanodine receptor (RyR2) has a critical role in controlling Ca2+ release from the
sarcoplasmic reticulum (SR) throughout the cardiac cycle. RyR2 protein has multiple …

Structural insights into Ca2+-activated long-range allosteric channel gating of RyR1

R Wei, X Wang, Y Zhang, S Mukherjee, L Zhang… - Cell research, 2016 - nature.com
Ryanodine receptors (RyRs) are a class of giant ion channels with molecular mass over 2.2
mega-Daltons. These channels mediate calcium signaling in a variety of cells. Since more …

[HTML][HTML] Ryanodine receptor-mediated arrhythmias and sudden cardiac death

LM Blayney, FA Lai - Pharmacology & therapeutics, 2009 - Elsevier
The cardiac ryanodine receptor-Ca2+ release channel (RyR2) is an essential sarcoplasmic
reticulum (SR) transmembrane protein that plays a central role in excitation–contraction …

Molecular and electrophysiological bases of catecholaminergic polymorphic ventricular tachycardia

U Mohamed, C Napolitano… - Journal of cardiovascular …, 2007 - Wiley Online Library
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited
arrhythmogenic disorder characterized by adrenergically mediated polymorphic ventricular …

Disease mutations in the ryanodine receptor N-terminal region couple to a mobile intersubunit interface

L Kimlicka, K Lau, CC Tung, F Van Petegem - Nature communications, 2013 - nature.com
Ryanodine receptors are large channels that release Ca2+ from the endoplasmic and
sarcoplasmic reticulum. Hundreds of RyR mutations can cause cardiac and skeletal muscle …

Ryanodine receptor structure: progress and challenges

SL Hamilton, II Serysheva - Journal of Biological Chemistry, 2009 - ASBMB
Ryanodine-sensitive Ca2 release channels, also known as the ryanodine receptors (RyRs),
2 are homotetramers of an 550-kDa subunit (Fig. 1) that are resident proteins of intracellular …

Cardiac and skeletal muscle disorders caused by mutations in the intracellular Ca2+ release channels

SG Priori, C Napolitano - The Journal of clinical …, 2005 - Am Soc Clin Investig
Here we review the current knowledge about the mutations of the gene encoding the cardiac
ryanodine receptor (RyR2) that cause cardiac arrhythmias. Similarities between the …

Ryanodine receptor regulation by intramolecular interaction between cytoplasmic and transmembrane domains

CH George, H Jundi, NL Thomas… - Molecular biology of …, 2004 - Am Soc Cell Biol
Ryanodine receptors (RyR) function as Ca2+ channels that regulate Ca2+ release from
intracellular stores to control a diverse array of cellular processes. The massive cytoplasmic …

The role of the S4-S5 linker and C-terminal tail in inositol 1, 4, 5-trisphosphate receptor function

ZT Schug, SK Joseph - Journal of Biological Chemistry, 2006 - ASBMB
In previous studies we have suggested that spatial proximity of the C-and N-terminal
domains of inositol 1, 4, 5-trisphosphate receptors (IP 3 Rs) may be critical for the channel …