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Hypernitrosylated ryanodine receptor/calcium release channels are leaky in dystrophic muscle
Duchenne muscular dystrophy is characterized by progressive muscle weakness and early death resulting from dystrophin deficiency. Loss of dystrophin results in disruption of a large dystrophinExpand
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Ryanodine receptor oxidation causes intracellular calcium leak and muscle weakness in aging.
Age-related loss of muscle mass and force (sarcopenia) contributes to disability and increased mortality. Ryanodine receptor 1 (RyR1) is the skeletal muscle sarcoplasmic reticulum calcium releaseExpand
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Rapidly progressive diaphragmatic weakness and injury during mechanical ventilation in humans.
RATIONALE Diaphragmatic function is a major determinant of the ability to successfully wean patients from mechanical ventilation (MV). Paradoxically, MV itself results in a rapid loss ofExpand
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Remodeling of ryanodine receptor complex causes “leaky” channels: A molecular mechanism for decreased exercise capacity
During exercise, defects in calcium (Ca2+) release have been proposed to impair muscle function. Here, we show that during exercise in mice and humans, the major Ca2+ release channel required forExpand
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Osteocalcin Signaling in Myofibers Is Necessary and Sufficient for Optimum Adaptation to Exercise.
Circulating levels of undercarboxylated and bioactive osteocalcin double during aerobic exercise at the time levels of insulin decrease. In contrast, circulating levels of osteocalcin plummet earlyExpand
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Leaky RyR2 trigger ventricular arrhythmias in Duchenne muscular dystrophy
Patients with Duchenne muscular dystrophy (DMD) have a progressive dilated cardiomyopathy associated with fatal cardiac arrhythmias. Electrical and functional abnormalities have been attributed toExpand
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PKA phosphorylation activates the calcium release channel (ryanodine receptor) in skeletal muscle
The type 1 ryanodine receptor (RyR1) on the sarcoplasmic reticulum (SR) is the major calcium (Ca2+) release channel required for skeletal muscle excitation–contraction (EC) coupling. RyR1 function isExpand
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AMPK activation stimulates autophagy and ameliorates muscular dystrophy in the mdx mouse diaphragm.
Duchenne muscular dystrophy (DMD) is characterized by myofiber death from apoptosis or necrosis, leading in many patients to fatal respiratory muscle weakness. Among other pathological features, DMDExpand
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Length and protein kinase A modulations of myocytes in cardiac myosin binding protein C-deficient mice.
OBJECTIVE Beta-adrenergic stimulation modulates cardiac contractility through protein kinase A (PKA), which phosphorylates proteins such as troponin I (cTnI) and C-protein (cMyBP-C). The relativeExpand
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Absence of triadin, a protein of the calcium release complex, is responsible for cardiac arrhythmia with sudden death in human
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited arrhythmogenic disease so far related to mutations in the cardiac ryanodine receptor (RYR2) or the cardiac calsequestrinExpand
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