Cardiac ryanodine receptor calcium release deficiency syndrome
نویسندگان
چکیده
Clinical and basic studies shed light on the mechanism, diagnosis, treatment of cardiac ryanodine receptor Ca 2+ release deficiency syndrome.
منابع مشابه
Ryanodine receptor calcium release channels.
The ryanodine receptors (RyRs) are a family of Ca2+ release channels found on intracellular Ca2+ storage/release organelles. The RyR channels are ubiquitously expressed in many types of cells and participate in a variety of important Ca2+ signaling phenomena (neurotransmission, secretion, etc.). In striated muscle, the RyR channels represent the primary pathway for Ca2+ release during the excit...
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Aberrant Zn(2+) homeostasis is a hallmark of certain cardiomyopathies associated with altered contractile force. In this study, we addressed whether Zn(2+) modulates cardiac ryanodine receptor gating and Ca(2+) dynamics in isolated cardiomyocytes. We reveal that Zn(2+) is a high affinity regulator of RyR2 displaying three modes of operation. Picomolar free Zn(2+) concentrations potentiate RyR2 ...
متن کاملClinical implications of cardiac ryanodine receptor/calcium release channel mutations linked to sudden cardiac death.
The cardiac ryanodine receptor (RyR2) is the major calcium (Ca ) release channel on the sarcoplasmic reticulum (SR) in cardiomyocytes. During excitationcontraction, coupling intracellular Ca stored in the SR is released via RyR2 to activate muscle contraction. In the heart, excitation-contraction coupling is activated by Ca influx via the L-type Ca channel that activates RyR2, a process referre...
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Background— -Adrenergic receptor blockade is one of the most effective treatments for heart failure, a leading cause of mortality worldwide. The use of -adrenergic receptor blockers in patients with heart failure is counterintuitive, however, because they are known to decrease contractility in normal hearts. The ryanodine receptor (RyR2) on cardiac sarcoplasmic reticulum is the key calcium rele...
متن کاملFKBP12.6 Deficiency and Defective Calcium Release Channel (Ryanodine Receptor) Function Linked to Exercise-Induced Sudden Cardiac Death
Arrhythmias, a common cause of sudden cardiac death, can occur in structurally normal hearts, although the mechanism is not known. In cardiac muscle, the ryanodine receptor (RyR2) on the sarcoplasmic reticulum releases the calcium required for muscle contraction. The FK506 binding protein (FKBP12.6) stabilizes RyR2, preventing aberrant activation of the channel during the resting phase of the c...
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ژورنال
عنوان ژورنال: Science Translational Medicine
سال: 2021
ISSN: ['1946-6242', '1946-6234']
DOI: https://doi.org/10.1126/scitranslmed.aba7287