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 …

Orai1 inhibitors as potential treatments for pulmonary arterial hypertension

B Masson, H Le Ribeuz, J Sabourin, L Laubry… - Circulation …, 2022 - Am Heart Assoc
Background: Pulmonary arterial hypertension (PAH) is characterized by progressive distal
pulmonary artery (PA) obstruction, leading to right ventricular hypertrophy and failure …

The builders of the junction: roles of junctophilin1 and junctophilin2 in the assembly of the sarcoplasmic reticulum–plasma membrane junctions in striated muscle

S Perni - Biomolecules, 2022 - mdpi.com
Contraction of striated muscle is triggered by a massive release of calcium from the
sarcoplasmic reticulum (SR) into the cytoplasm. This intracellular calcium release is initiated …

Heart failure in mice induces a dysfunction of the sinus node associated with reduced CaMKII signaling

JB Xue, A Val-Blasco, M Davoodi, S Gómez… - Journal of General …, 2022 - rupress.org
Dysfunction of the sinoatrial node (SAN), the natural heart pacemaker, is common in heart
failure (HF) patients. SAN spontaneous activity relies on various ion currents in the plasma …

Diversity of cells and signals in the cardiovascular system

E Grandi, MF Navedo, JJ Saucerman… - The Journal of …, 2023 - Wiley Online Library
This white paper is the outcome of the seventh UC Davis Cardiovascular Research
Symposium on Systems Approach to Understanding Cardiovascular Disease and …

Kir2. 1 dysfunction at the sarcolemma and the sarcoplasmic reticulum causes arrhythmias in a mouse model of Andersen–Tawil syndrome type 1

Á Macías, A González-Guerra… - Nature Cardiovascular …, 2022 - nature.com
Andersen–Tawil syndrome type 1 (ATS1) is associated with life-threatening arrhythmias of
unknown mechanism. In this study, we generated and characterized a mouse model of …

Sarcoplasmic reticulum Ca2+ dysregulation in the pathophysiology of inherited arrhythmia: an update

Y Du, LJ Demillard, J Ren - Biochemical Pharmacology, 2022 - Elsevier
Inherited arrhythmias are the leading causes for cardiac arrest and sudden cardiac death
(SCD). Other than ion channel mutations, inherited arrhythmias including catecholaminergic …

Local recovery of cardiac calcium‐induced calcium release interrogated by ultra‐effective, two‐photon uncaging of calcium

R Janicek, H Agarwal, AM Gómez… - The Journal of …, 2021 - Wiley Online Library
Key points In cardiac myocytes, subcellular local calcium release signals, calcium sparks,
are recruited to form each cellular calcium transient and activate the contractile machinery …

Using hiPSC‐CMs to examine mechanisms of catecholaminergic polymorphic ventricular tachycardia

A Arslanova, S Shafaattalab, K Ye, P Asghari… - Current …, 2021 - Wiley Online Library
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a potentially lethal
inherited cardiac arrhythmia condition, triggered by physical or acute emotional stress, that …

[HTML][HTML] Uptake-leak balance of SR Ca2+ determines arrhythmogenic potential of RyR2R420Q+/− cardiomyocytes

R Lopez, R Janicek, M Fernandez-Tenorio… - Journal of molecular and …, 2022 - Elsevier
Mutations of the RyR2 are channelopathies that can predispose to life threatening
catecholaminergic polymorphic ventricular tachycardias (CPVTs) during exercise or stress …