Ryanodine receptor dysfunction causes senescence and fibrosis in Duchenne dilated cardiomyopathy
… and cardiac fibrosis are induced by SR Ca2+ leak via RyR2 in … in the context of Duchenne
muscular dystrophy (DMD). … Short-coupled polymorphic ventricular tachycardia at rest …
muscular dystrophy (DMD). … Short-coupled polymorphic ventricular tachycardia at rest …
Sarcoplasmic reticulum Ca2+ dysregulation in the pathophysiology of inherited arrhythmia: an update
Y Du, LJ Demillard, J Ren - Biochemical Pharmacology, 2022 - Elsevier
… gene mutations in inherited cardiac arrhythmias, in an effort … in a mouse model of Duchenne
muscular dystrophy (DMD), … of RyR2 and elevated SR Ca 2+ -leak en route to CPVT-like …
muscular dystrophy (DMD), … of RyR2 and elevated SR Ca 2+ -leak en route to CPVT-like …
[HTML][HTML] Skeletal ryanodine receptors are involved in impaired myogenic differentiation in Duchenne muscular dystrophy patients
P Meyer, C Notarnicola, AC Meli, S Matecki… - International Journal of …, 2021 - mdpi.com
… Leaky RYR1 channels could be implicated in various … Furthermore, we reported that cardiac
arrhythmias in mdx mice can be prevented by stabilizing cardiomyocytes RYR2 channels …
arrhythmias in mdx mice can be prevented by stabilizing cardiomyocytes RYR2 channels …
Ryanodine receptor remodeling in cardiomyopathy and muscular dystrophy caused by lamin A/C gene mutation
H Dridi, W Wu, SR Reiken, RM Ofer… - Human Molecular …, 2020 - academic.oup.com
… remodeling of RyR2 in the heart and RyR1 in skeletal muscle. … SR Ca 2+ release via RyR2
in cardiomyocytes is triggered … S107 which fixes the channel leak. S107 prevents calstabin …
in cardiomyocytes is triggered … S107 which fixes the channel leak. S107 prevents calstabin …
[HTML][HTML] Targeting ryanodine receptors to treat human diseases
AR Marks - The Journal of Clinical Investigation, 2023 - Am Soc Clin Investig
… Ca 2+ leak was linked to ventricular arrhythmias (3). RyR2s are … Duchenne muscular dystrophy
and limb-girdle muscular … Following the discovery of the leaky RyR2 in failing hearts, we …
and limb-girdle muscular … Following the discovery of the leaky RyR2 in failing hearts, we …
[HTML][HTML] The multifaceted view of heart problem in Duchenne muscular dystrophy
U Florczyk-Soluch, K Polak, J Dulak - Cellular and Molecular Life Sciences, 2021 - Springer
… related to ongoing muscle damage and the leakage of myofiber … A particular risk is carried
by ventricular arrhythmias, which … Patients with HF are at high risk of ventricular arrhythmia and …
by ventricular arrhythmias, which … Patients with HF are at high risk of ventricular arrhythmia and …
[HTML][HTML] Dysregulation of calcium handling in duchenne muscular dystrophy-associated dilated cardiomyopathy: mechanisms and experimental therapeutic strategies
ML Law, H Cohen, AA Martin, ABB Angulski… - Journal of clinical …, 2020 - mdpi.com
… cardiac wall thickness, and cardiac arrhythmias [3,23]. … The initial calcium influx then triggers
a larger calcium release … of ROS and increased RyR2 calcium leak. Taken together, these …
a larger calcium release … of ROS and increased RyR2 calcium leak. Taken together, these …
Intracellular calcium leak as a therapeutic target for RYR1-related myopathies
… function in Duchenne muscular dystrophy [5, 14], … fatal hypermetabolic response to
pharmacological triggers, is not well … by mutations in the cardiac ryanodine receptor (RyR2). Due …
pharmacological triggers, is not well … by mutations in the cardiac ryanodine receptor (RyR2). Due …
Stabilizing ryanodine receptors improves left ventricular function in juvenile dogs with Duchenne muscular dystrophy
… triggering of cardiac arrhythmias and remodeling in DMD (5). Inhibiting the depletion of
FKBP12.6 from the RyR2 … ) stops the SR Ca 2+ leak, controls aberrant depolarization in isolated …
FKBP12.6 from the RyR2 … ) stops the SR Ca 2+ leak, controls aberrant depolarization in isolated …
Potential therapeutic targets for hypotension in duchenne muscular dystrophy
H Saxena, NL Weintraub, Y Tang - Medical Hypotheses, 2024 - Elsevier
… We hypothesize that heightened KCNQ5 and RyR2 expression … RyR2 inhibitor, thereby
reducing calcium leakage through … agent for managing ventricular arrhythmias in DMD patients. …
reducing calcium leakage through … agent for managing ventricular arrhythmias in DMD patients. …