Fixing ryanodine receptor Ca2+ leak–a novel therapeutic strategy for contractile failure in heart and skeletal muscle

DC Andersson, AR Marks - Drug discovery today: disease mechanisms, 2010 - Elsevier
A crucial component in regulating cardiac and skeletal muscles contractility is the release of
Ca2+ via ryanodine receptor (RyR) Ca2+ release channels in the sarcoplasmic reticulum …

[HTML][HTML] Posttranslational modifications of cardiac ryanodine receptors: Ca2+ signaling and EC-coupling

E Niggli, ND Ullrich, D Gutierrez, S Kyrychenko… - … et Biophysica Acta (BBA …, 2013 - Elsevier
In cardiac muscle, a number of posttranslational protein modifications can alter the function
of the Ca2+ release channel of the sarcoplasmic reticulum (SR), also known as the …

Physiology and pathophysiology of excitation–contraction coupling: the functional role of ryanodine receptor

G Santulli, DR Lewis, AR Marks - Journal of muscle research and cell …, 2017 - Springer
Abstract Calcium (Ca 2+) release from intracellular stores plays a key role in the regulation
of skeletal muscle contraction. The type 1 ryanodine receptors (RyR1) is the major Ca 2+ …

Uniting the divergent Wolfram syndrome–linked proteins WFS1 and CISD2 as modulators of Ca2+ signaling

J Loncke, T Vervliet, JB Parys, A Kaasik, G Bultynck - Science Signaling, 2021 - science.org
Mutations in WFS1 (which encodes Wolframin, WFS1) and CISD2 (which encodes CDGSH
iron sulfur domain 2) result in Wolfram syndrome (WS), a rare genetic disorder that starts …

The role of calcium handling mechanisms in reperfusion injury

K Pittas, DA Vrachatis, C Angelidis… - Current …, 2018 - ingentaconnect.com
Cardiovascular diseases, such as stroke and myocardial infarction (MI) remain the major
cause of death and disability worldwide. However, the mortality of MI has declined …

Bioenergetic and metabolic impairments in induced pluripotent stem cell-derived cardiomyocytes generated from Duchenne muscular dystrophy patients

L Willi, I Abramovich, J Fernandez-Garcia… - International journal of …, 2022 - mdpi.com
Duchenne muscular dystrophy (DMD) is caused by mutations in the dystrophin gene and
dilated cardiomyopathy (DCM) is a major cause of morbidity and mortality in DMD patients …

Evolution and developmental functions of the dystrophin-associated protein complex: Beyond the idea of a muscle-specific cell adhesion complex

V Mirouse - Frontiers in cell and developmental biology, 2023 - frontiersin.org
The Dystrophin-Associated Protein Complex (DAPC) is a well-defined and evolutionarily
conserved complex in animals. DAPC interacts with the F-actin cytoskeleton via dystrophin …

Leaky ryanodine receptors in β-sarcoglycan deficient mice: a potential common defect in muscular dystrophy

DC Andersson, AC Meli, S Reiken, MJ Betzenhauser… - Skeletal muscle, 2012 - Springer
Background Disruption of the sarcolemma-associated dystrophin-glycoprotein complex
underlies multiple forms of muscular dystrophy, including Duchenne muscular dystrophy …

Cardiac Piezo1 Exacerbates Lethal Ventricular Arrhythmogenesis by Linking Mechanical Stress with Ca2+ Handling After Myocardial Infarction

S Su, Y Zhang, W Li, Y Xi, Y Lu, J Shen, Y Ma, Y Wang… - Research, 2023 - spj.science.org
Ventricular arrhythmogenesis is a key cause of sudden cardiac death following myocardial
infarction (MI). Accumulating data show that ischemia, sympathetic activation, and …

RyR2-targeting therapy prevents left ventricular remodeling and ventricular tachycardia in post-infarction heart failure

S Fujii, S Kobayashi, Y Chang, J Nawata… - Journal of Molecular and …, 2023 - Elsevier
Background Dantrolene binds to the Leu 601-Cys 620 region of the N-terminal domain of
cardiac ryanodine receptor (RyR2), which corresponds to the Leu 590-Cys 609 region of the …