L-Type Ca2+ Channel Function During Timothy Syndrome
نویسندگان
چکیده
منابع مشابه
Restoration of normal L-type Ca2+ channel function during Timothy syndrome by ablation of an anchoring protein.
RATIONALE L-type Ca(2+) (Ca(V)1.2) channels shape the cardiac action potential waveform and are essential for excitation-contraction coupling in heart. A gain-of-function G406R mutation in a cytoplasmic loop of Ca(V)1.2 channels causes long QT syndrome 8 (LQT8), a disease also known as Timothy syndrome. However, the mechanisms by which this mutation enhances Ca(V)1.2-LQT8 currents and generates...
متن کاملIncreased coupled gating of L-type Ca2+ channels during hypertension and Timothy syndrome.
RATIONALE L-Type (Cav1.2) Ca(2+) channels are critical regulators of muscle and neural function. Although Cav1.2 channel activity varies regionally, little is known about the mechanisms underlying this heterogeneity. OBJECTIVE To test the hypothesis that Cav1.2 channels can gate coordinately. METHODS AND RESULTS We used optical and electrophysiological approaches to record Cav1.2 channel ac...
متن کاملBrief UltraRapid Communication Restoration of Normal L-Type Ca Channel Function During Timothy Syndrome by Ablation of an Anchoring Protein
Rationale: L-type Ca (CaV1.2) channels shape the cardiac action potential waveform and are essential for excitation–contraction coupling in heart. A gain-of-function G406R mutation in a cytoplasmic loop of CaV1.2 channels causes long QT syndrome 8 (LQT8), a disease also known as Timothy syndrome. However, the mechanisms by which this mutation enhances CaV1.2-LQT8 currents and generates lethal a...
متن کاملIncreased Coupled Gating of L-Type Ca Channels During Hypertension and Timothy Syndrome
Rationale: L-Type (Cav1.2) Ca channels are critical regulators of muscle and neural function. Although Cav1.2 channel activity varies regionally, little is known about the mechanisms underlying this heterogeneity. Objective: To test the hypothesis that Cav1.2 channels can gate coordinately. Methods and Results: We used optical and electrophysiological approaches to record Cav1.2 channel activit...
متن کاملA sympathetic model of L-type Ca2+ channel-triggered arrhythmias.
L-TYPE CA2 CHANNELS PLAY a pivotal role in regulating cellular excitability and contractility in cardiac tissue. Ca influx through these channels [L-type Ca current (ICa,L)] contributes to the plateau phase of the ventricular action potential and initiates excitation-contraction coupling by activating Ca release channels or ryanodine receptors in the sarcoplasmic reticulum (1). The resulting Ca...
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ژورنال
عنوان ژورنال: Trends in Cardiovascular Medicine
سال: 2012
ISSN: 1050-1738
DOI: 10.1016/j.tcm.2012.06.015