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

No abstract is available for this paper.

• Publication year

  2015

• Venue

  Cardiovascular Research

• Publication date

  Unknown publication date

• Fields of study

  Biology, Medicine

• Identifiers
  DOI 10.1093/cvr/cvu240PMID 25411383
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

• N-terminus oligomerization is conserved in intracellular calcium release channels

  2014influential reference
• N-terminus oligomerization regulates the function of cardiac ryanodine receptors

  2013influential reference
• Disease mutations in the ryanodine receptor N-terminal region couple to a mobile intersubunit interface

  2013cited by this paper
• Calcium cycling proteins and heart failure: mechanisms and therapeutics.

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• 'Ryanopathy': causes and manifestations of RyR2 dysfunction in heart failure.

  2013cited by this paper
• The cardiac ryanodine receptor N-terminal region contains an anion binding site that is targeted by disease mutations.

  2013influential reference
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  2012cited by this paper
• Calcium Leak Through Ryanodine Receptors Leads to Atrial Fibrillation in 3 Mouse Models of Catecholaminergic Polymorphic Ventricular Tachycardia

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• Dantrolene prevents arrhythmogenic Ca2+ release in heart failure.

  2012cited by this paper
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  2011cited by this paper
• The pathobiology of arrhythmogenic cardiomyopathy.

  2011cited by this paper
• Arrhythmogenic right ventricular cardiomyopathy

  2011cited by this paper
• Localization of the Dantrolene-binding Sequence near the FK506-binding Protein-binding Site in the Three-dimensional Structure of the Ryanodine Receptor*

  2011cited by this paper
• Catecholaminergic Polymorphic Ventricular Tachycardia Is Caused by Mutation-Linked Defective Conformational Regulation of the Ryanodine Receptor

  2010influential reference
• Dantrolene, a therapeutic agent for malignant hyperthermia, inhibits catecholaminergic polymorphic ventricular tachycardia in a RyR2(R2474S/+) knock-in mouse model.

  2010cited by this paper
• Arrhythmogenic right ventricular cardiomyopathy

  2009cited by this paper
• Ryanodine receptor-mediated arrhythmias and sudden cardiac death

  2009cited by this paper
• Dantrolene, a therapeutic agent for malignant hyperthermia, markedly improves the function of failing cardiomyocytes by stabilizing interdomain interactions within the ryanodine receptor.

  2009influential reference
• Mechanisms of Disease: molecular genetics of arrhythmogenic right ventricular dysplasia/cardiomyopathy

  2008cited by this paper
• Ryanodine receptor structure, function and pathophysiology

  2007cited by this paper
• Calcium : a matter of life or death

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• Loss of luminal Ca2+ activation in the cardiac ryanodine receptor is associated with ventricular fibrillation and sudden death

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• Probing a putative dantrolene-binding site on the cardiac ryanodine receptor.

  2005cited by this paper
• Enhanced Store Overload–Induced Ca2+ Release and Channel Sensitivity to Luminal Ca2+ Activation Are Common Defects of RyR2 Mutations Linked to Ventricular Tachycardia and Sudden Death

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• Arrhythmogenic Mutation-Linked Defects in Ryanodine Receptor Autoregulation Reveal a Novel Mechanism of Ca2+ Release Channel Dysfunction

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• Spectrum and prevalence of cardiac ryanodine receptor (RyR2) mutations in a cohort of unrelated patients referred explicitly for long QT syndrome genetic testing.

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• Juvenile sudden death in a family with polymorphic ventricular arrhythmias caused by a novel RyR2 gene mutation: evidence of specific morphological substrates.

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• Dantrolene – A review of its pharmacology, therapeutic use and new developments

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• RyR 2 mutations linked to ventricular tachycardia and sudden death reduce the threshold for store-overload-induced Ca 2 release ( SOICR )

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• Sudden Death in Familial Polymorphic Ventricular Tachycardia Associated With Calcium Release Channel (Ryanodine Receptor) Leak

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• RyR2 mutations linked to ventricular tachycardia and sudden death reduce the threshold for store-overload-induced Ca2+ release (SOICR).

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• FKBP12.6 deficiency and defective calcium release channel (ryanodine receptor) function linked to exercise-induced sudden cardiac death.

  2003cited by this paper
• Oligomerization of the cardiac ryanodine receptor C-terminal tail.

  2003cited by this paper
• Dysregulated Ryanodine Receptors Mediate Cellular Toxicity

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• Sarcoplasmic reticulum Ca2+ and heart failure: roles of diastolic leak and Ca2+ transport.

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• Ryanodine Receptor Mutations Associated With Stress-Induced Ventricular Tachycardia Mediate Increased Calcium Release in Stimulated Cardiomyocytes

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• Calcium cycling in congestive heart failure.

  2002cited by this paper
• Identification of a Dantrolene-binding Sequence on the Skeletal Muscle Ryanodine Receptor*

  2002cited by this paper
• Enhanced Basal Activity of a Cardiac Ca2+ Release Channel (Ryanodine Receptor) Mutant Associated With Ventricular Tachycardia and Sudden Death

  2002cited by this paper
• Dantrolene Inhibition of Ryanodine Receptor Ca2+Release Channels

  2001cited by this paper
• Identification of mutations in the cardiac ryanodine receptor gene in families affected with arrhythmogenic right ventricular cardiomyopathy type 2 (ARVD2).

  2001cited by this paper
• Mutations in the Cardiac Ryanodine Receptor Gene (hRyR2) Underlie Catecholaminergic Polymorphic Ventricular Tachycardia

  2001cited by this paper
• Evidence for a role of C‐terminal amino acid residues in skeletal muscle Ca2+ release channel (ryanodine receptor) function

  1997cited by this paper
• The pharmacology of ryanodine and related compounds.

  1997cited by this paper
• Functional calcium release channel formed by the carboxyl-terminal portion of ryanodine receptor.

  1997cited by this paper
• Caffeine-induced Release of Intracellular Ca2+ from Chinese Hamster Ovary Cells Expressing Skeletal Muscle Ryanodine Receptor

  1997cited by this paper
• Interaction between Ryanodine and Neomycin Binding Sites on Ca Release Channel from Skeletal Muscle Sarcoplasmic Reticulum (*)

  1996cited by this paper

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  2025cites this paper
• Crosstalk between Ryanodine Receptor 2 Dysfunction and FK506-Binding Proteins in Pulmonary Hypertension.

  2025cites this paper
• Disparate molecular mechanisms in cardiac ryanodine receptor channelopathies

  2024cites this paper
• Molecular Aspects Implicated in Dantrolene Selectivity with Respect to Ryanodine Receptor Isoforms

  2023cites this paper
• Z16b, a natural compound from Ganoderma cochlear is a novel RyR2 stabilizer preventing catecholaminergic polymorphic ventricular tachycardia

  2022cites this paper
• Defective ryanodine receptor N-terminus inter-subunit interaction is a common mechanism in neuromuscular and cardiac disorders

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• Molecular, Subcellular, and Arrhythmogenic Mechanisms in Genetic RyR2 Disease

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• Impaired Binding to Junctophilin-2 and Nanostructural Alteration in CPVT Mutation

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  2018cites this paper
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