Dantrolene rescues arrhythmogenic RYR2 defect in a patient-specific stem cell model of catecholaminergic polymorphic ventricular tachycardia

Inherited Dysfunction of Sarcoplasmic Reticulum Ca 2+ Handling and Arrhythmogenesis

(2011) Silvia G. Priori et al.   CIRCULATION RESEARCH

Localization of the Dantrolene-binding Sequence near the FK506-binding Protein-binding Site in the Three-dimensional Structure of the Ryanodine Receptor

(2011) Ruiwu Wang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Using induced pluripotent stem cells to investigate cardiac phenotypes in Timothy syndrome

(2011) Masayuki Yazawa et al.   NATURE

Investigating monogenic and complex diseases with pluripotent stem cells

(2011) Hao Zhu et al.   NATURE REVIEWS GENETICS

Global transcriptional profiles of beating clusters derived from human induced pluripotent stem cells and embryonic stem cells are highly similar

(2010) Manoj K Gupta et al.   BMC DEVELOPMENTAL BIOLOGY

Dantrolene, a Therapeutic Agent for Malignant Hyperthermia, Inhibits Catecholaminergic Polymorphic Ventricular Tachycardia in a RyR2R2474S/+ Knock-In Mouse Model

(2010) Shigeki Kobayashi et al.   CIRCULATION JOURNAL

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

(2010) Hitoshi Uchinoumi et al.   CIRCULATION RESEARCH

Kinetics of FKBP12.6 Binding to Ryanodine Receptors in Permeabilized Cardiac Myocytes and Effects on Ca Sparks

(2010) Tao Guo et al.   CIRCULATION RESEARCH

Ryanodine receptor mutations in arrhythmia: The continuing mystery of channel dysfunction

(2010) N. Lowri Thomas et al.   FEBS LETTERS

Dynamic, inter-subunit interactions between the N-terminal and central mutation regions of cardiac ryanodine receptor

(2010) Z. Liu et al.   JOURNAL OF CELL SCIENCE

The amino-terminal disease hotspot of ryanodine receptors forms a cytoplasmic vestibule

(2010) Ching-Chieh Tung et al.   NATURE

Patient-specific induced pluripotent stem-cell-derived models of LEOPARD syndrome

(2010) Xonia Carvajal-Vergara et al.   NATURE

Patient-Specific Induced Pluripotent Stem-Cell Models for Long-QT Syndrome

(2010) Alessandra Moretti et al.   NEW ENGLAND JOURNAL OF MEDICINE

Ryanodine Receptors: Structure, Expression, Molecular Details, and Function in Calcium Release

(2010) J. T. Lanner et al.   Cold Spring Harbor Perspectives in Biology

From the Ryanodine Receptor to Cardiac Arrhythmias

(2009) D. A. Eisner et al.   CIRCULATION JOURNAL

Mouse and human induced pluripotent stem cells as a source for multipotent Isl1+ cardiovascular progenitors

(2009) Alessandra Moretti et al.   FASEB JOURNAL

Mechanisms and clinical management of inherited channelopathies: Long QT syndrome, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia, and short QT syndrome

(2009) Elizabeth S. Kaufman   HEART RHYTHM

Dantrolene, a Therapeutic Agent for Malignant Hyperthermia, Markedly Improves the Function of Failing Cardiomyocytes by Stabilizing Interdomain Interactions Within the Ryanodine Receptor

(2009) Shigeki Kobayashi et al.   JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY

Reversing Advanced Heart Failure by Targeting Ca2+ Cycling

(2008) David M. Kaye et al.   Annual Review of Medicine

Defective domain-domain interactions within the ryanodine receptor as a critical cause of diastolic Ca2+ leak in failing hearts

(2008) H. Tateishi et al.   CARDIOVASCULAR RESEARCH

Ryanodine receptor and calsequestrin in arrhythmogenesis: What we have learnt from genetic diseases and transgenic mice

(2008) Nian Liu et al.   JOURNAL OF MOLECULAR AND CELLULAR CARDIOLOGY