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 …
sarcoplasmic reticulum (SR) throughout the cardiac cycle. RyR2 protein has multiple …
“Ryanopathies” and RyR2 dysfunctions: can we further decipher them using in vitro human disease models?
The regulation of intracellular calcium (Ca2+) homeostasis is fundamental to maintain
normal functions in many cell types. The ryanodine receptor (RyR), the largest intracellular …
normal functions in many cell types. The ryanodine receptor (RyR), the largest intracellular …
Impaired binding to junctophilin-2 and nanostructural alteration in CPVT mutation
L Yin, A Zahradnikova Jr, R Rizzetto… - Circulation …, 2021 - Am Heart Assoc
Rationale: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare disease,
manifested by syncope or sudden death in children or young adults under stress conditions …
manifested by syncope or sudden death in children or young adults under stress conditions …
CaMKII-dependent phosphorylation of RyR2 promotes targetable pathological RyR2 conformational shift
H Uchinoumi, Y Yang, T Oda, N Li, KM Alsina… - Journal of molecular and …, 2016 - Elsevier
Diastolic calcium (Ca) leak via cardiac ryanodine receptors (RyR2) can cause arrhythmias
and heart failure (HF). Ca/calmodulin (CaM)-dependent kinase II (CaMKII) is upregulated …
and heart failure (HF). Ca/calmodulin (CaM)-dependent kinase II (CaMKII) is upregulated …
Molecular aspects implicated in dantrolene selectivity with respect to ryanodine receptor isoforms
J Gaburjakova, M Gaburjakova - International Journal of Molecular …, 2023 - mdpi.com
Dantrolene is an intra-cellularly acting skeletal muscle relaxant used for the treatment of the
rare genetic disorder, malignant hyperthermia (MH). In most cases, MH susceptibility is …
rare genetic disorder, malignant hyperthermia (MH). In most cases, MH susceptibility is …
Calsequestrin interacts directly with the cardiac ryanodine receptor luminal domain
A Handhle, CE Ormonde, NL Thomas… - Journal of cell …, 2016 - journals.biologists.com
Cardiac muscle contraction requires sarcoplasmic reticulum (SR) Ca2+ release mediated by
the quaternary complex comprising the ryanodine receptor 2 (RyR2), calsequestrin 2 …
the quaternary complex comprising the ryanodine receptor 2 (RyR2), calsequestrin 2 …
Cardiac ryanodine receptor N-terminal region biosensors identify novel inhibitors via FRET-based high-throughput screening
J Zhang, DP Singh, CY Ko, R Nikolaienko… - Journal of Biological …, 2022 - ASBMB
The N-terminal region (NTR) of ryanodine receptor (RyR) channels is critical for the
regulation of Ca 2+ release during excitation–contraction (EC) coupling in muscle. The NTR …
regulation of Ca 2+ release during excitation–contraction (EC) coupling in muscle. The NTR …
Z16b, a natural compound from Ganoderma cochlear is a novel RyR2 stabilizer preventing catecholaminergic polymorphic ventricular tachycardia
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited, lethal
ventricular arrhythmia triggered by catecholamines. Mutations in genes that encode cardiac …
ventricular arrhythmia triggered by catecholamines. Mutations in genes that encode cardiac …
Identification of an amino-terminus determinant critical for ryanodine receptor/Ca2+ release channel function
M Seidel, CR de Meritens, L Johnson… - Cardiovascular …, 2021 - academic.oup.com
Aims The cardiac ryanodine receptor (RyR2), which mediates intracellular Ca2+ release to
trigger cardiomyocyte contraction, participates in development of acquired and inherited …
trigger cardiomyocyte contraction, participates in development of acquired and inherited …
Defective ryanodine receptor N-terminus inter-subunit interaction is a common mechanism in neuromuscular and cardiac disorders
Y Zhang, C Rabesahala de Meritens… - Frontiers in …, 2022 - frontiersin.org
The ryanodine receptor (RyR) is a homotetrameric channel mediating sarcoplasmic
reticulum Ca2+ release required for skeletal and cardiac muscle contraction. Mutations in …
reticulum Ca2+ release required for skeletal and cardiac muscle contraction. Mutations in …