Impaired Binding to Junctophilin2 and Nanostructural Alteration in CPVT Mutation

Identification of an amino-terminus determinant critical for ryanodine receptor/Ca2+ release channel function

(2020) Monika Seidel et al.   CARDIOVASCULAR RESEARCH

Loss of SPEG Inhibitory Phosphorylation of RyR2 Promotes Atrial Fibrillation

(2020) Hannah M. Campbell et al.   CIRCULATION

Progression of excitation-contraction coupling defects in doxorubicin cardiotoxicity

(2018) Anna Llach et al.   JOURNAL OF MOLECULAR AND CELLULAR CARDIOLOGY

β-Adrenergic induced SR Ca 2+ leak is mediated by an Epac-NOS pathway

(2017) Laëtitia Pereira et al.   JOURNAL OF MOLECULAR AND CELLULAR CARDIOLOGY

Real-time intra-store confocal Ca 2+ imaging in isolated mouse cardiomyocytes

(2016) Miguel Fernandez-Tenorio et al.   CELL CALCIUM

Junctophilin-2 gene therapy rescues heart failure by normalizing RyR2-mediated Ca2+ release

(2016) Julia O. Reynolds et al.   INTERNATIONAL JOURNAL OF CARDIOLOGY

Junctophilin-2 in the nanoscale organisation and functional signalling of ryanodine receptor clusters in cardiomyocytes

(2016) Michelle L. Munro et al.   JOURNAL OF CELL SCIENCE

Structural basis for the gating mechanism of the type 2 ryanodine receptor RyR2

(2016) Wei Peng et al.   SCIENCE

Ryanodine receptor cluster fragmentation and redistribution in persistent atrial fibrillation enhance calcium release

(2015) Niall Macquaide et al.   CARDIOVASCULAR RESEARCH

Functional abnormalities in iPSC-derived cardiomyocytes generated from CPVT1 and CPVT2 patients carrying ryanodine or calsequestrin mutations

(2015) Atara Novak et al.   JOURNAL OF CELLULAR AND MOLECULAR MEDICINE

Reconciling depressed Ca2+sparks occurrence with enhanced RyR2 activity in failing mice cardiomyocytes

(2015) Gema Ruiz-Hurtado et al.   JOURNAL OF GENERAL PHYSIOLOGY

Fitting Linear Mixed-Effects Models Usinglme4

(2015) Douglas Bates et al.   Journal of Statistical Software

Arrhythmogenesis in a catecholaminergic polymorphic ventricular tachycardia mutation that depresses ryanodine receptor function

(2015) Yan-Ting Zhao et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

A knock-in mouse model of N-terminal R420W mutation of cardiac ryanodine receptor exhibits arrhythmogenesis with abnormal calcium dynamics in cardiomyocytes

(2014) Noriyuki Okudaira et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Dantrolene rescues aberrant N-terminus intersubunit interactions in mutant pro-arrhythmic cardiac ryanodine receptors

(2014) Monika Seidel et al.   CARDIOVASCULAR RESEARCH

Divergent Regulation of Ryanodine Receptor 2 Calcium Release Channels by Arrhythmogenic Human Calmodulin Missense Mutants

(2014) Hyun Seok Hwang et al.   CIRCULATION RESEARCH

A PKA-ezrin-Cx43 signaling complex controls gap junction communication and thereby trophoblast cell fusion

(2014) G. Pidoux et al.   JOURNAL OF CELL SCIENCE

Structural insights into the human RyR2 N-terminal region involved in cardiac arrhythmias

(2014) Ľubomír Borko et al.   ACTA CRYSTALLOGRAPHICA SECTION D-BIOLOGICAL CRYSTALLOGRAPHY

Control of Sarcoplasmic Reticulum Ca2+ Release by Stochastic RyR Gating within a 3D Model of the Cardiac Dyad and Importance of Induction Decay for CICR Termination

(2013) M.B. Cannell et al.   BIOPHYSICAL JOURNAL

Triadin regulates cardiac muscle couplon structure and microdomain Ca2+ signalling: a path towards ventricular arrhythmias

(2013) Nagesh Chopra et al.   CARDIOVASCULAR RESEARCH

Mutation E169K in Junctophilin-2 Causes Atrial Fibrillation Due to Impaired RyR2 Stabilization

(2013) David L. Beavers et al.   JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY

The Cardiac Ryanodine Receptor N-Terminal Region Contains an Anion Binding Site that Is Targeted by Disease Mutations

(2013) Lynn Kimlicka et al.   STRUCTURE

Paradoxical Effect of Increased Diastolic Ca 2+ Release and Decreased Sinoatrial Node Activity in a Mouse Model of Catecholaminergic Polymorphic Ventricular Tachycardia

(2012) Patricia Neco et al.   CIRCULATION

Heterogeneity of Ryanodine Receptor Dysfunction in a Mouse Model of Catecholaminergic Polymorphic Ventricular Tachycardia

(2012) Randall Loaiza et al.   CIRCULATION RESEARCH

Directed cardiomyocyte differentiation from human pluripotent stem cells by modulating Wnt/β-catenin signaling under fully defined conditions

(2012) Xiaojun Lian et al.   Nature Protocols

RyRCa2+ Leak Limits Cardiac Ca2+ Window Current Overcoming the Tonic Effect of Calmodulin in Mice

(2011) María Fernández-Velasco et al.   PLoS One

Catecholaminergic Polymorphic Ventricular Tachycardia Is Caused by Mutation-Linked Defective Conformational Regulation of the Ryanodine Receptor

(2010) Hitoshi Uchinoumi et al.   CIRCULATION RESEARCH

Ryanodol action on calcium sparks in ventricular myocytes

(2010) Josefina Ramos-Franco et al.   PFLUGERS ARCHIV-EUROPEAN JOURNAL OF PHYSIOLOGY

The RYR2-Encoded Ryanodine Receptor/Calcium Release Channel in Patients Diagnosed Previously With Either Catecholaminergic Polymorphic Ventricular Tachycardia or Genotype Negative, Exercise-Induced Long QT Syndrome

(2009) Argelia Medeiros-Domingo et al.   JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY

Increased Ca 2+ Sensitivity of the Ryanodine Receptor Mutant RyR2 R4496C Underlies Catecholaminergic Polymorphic Ventricular Tachycardia

(2008) María Fernández-Velasco et al.   CIRCULATION RESEARCH

Luminal Ca2+Regulation of Single Cardiac Ryanodine Receptors: Insights Provided by Calsequestrin and its Mutants

(2008) Jia Qin et al.   JOURNAL OF GENERAL PHYSIOLOGY