Short QT Syndrome: A Predictable Story

Brugada, Josép; Gussak, Ihor; Brugada, Pedro

doi:10.1159/000359995

Cited by 20 publications

(22 citation statements)

References 42 publications

(20 reference statements)

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“…6B and C) which accelerates the repolarization process and last produces a shorter QT interval (<360 ms) on an electrocardiogram (ECG). 23 So, the blockage to the outward currents of the Kir2.1 mutants is more significant. Then we moved to verify if styrax can block the outward currents permeated by Kir2.1 mutants, D172N or E299V.…”

Section: Resultsmentioning

confidence: 99%

“…Abnormal function of Kir2.1 will induce several diseases, such as Andersen syndrome [16][17][18][19][20][21] and SQT3 syndrome. [22][23][24] It has been identified that the gain function mutants, D172N 5,26,27 and E299V, 28 of Kir2.1 can cause SQT3 syndrome by enhancing the outward currents. However, there is no any clinical drug for SQT3 targeting at Kir2.1.…”

Section: Discussionmentioning

confidence: 99%

“…3 The abnormal function of Kir2.1 protein can cause the Andersen's syndrome (LQT type7) for the loss of function mutation [16][17][18][19][20][21] and type 3 short QT syndrome (SQT3) for the gain of function mutation. [22][23][24] SQT3 is characterized by abnormally short QT interval (<360 ms) in the electrocardiogram (ECG) with symptoms of atrial fibrillation, cardiac death, ventricular arrhythmias, syncopal event and palpitations. 25 The single point mutation of D172N, 5,26,27 E299V, 28 M301K, 29 K346T 30 of Kir2.1 will cause greater outward current (gain of function), and thus accelerate the repolarization process.…”

Section: Introductionmentioning

confidence: 99%

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Styrax blocks inward and outward current of Kir2.1 channel

Ren

Pang

et al. 2016

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Kir2.1 plays key roles in setting rest membrane potential and modulation of cell excitability. Mutations of Kir2.1, such as D172N or E299V, inducing gain-of-function, can cause type3 short QT syndrome (SQT3) due to the enlarged outward currents. So far, there is no clinical drug target to block the currents of Kir2.1. Here, we identified a novel blocker of Kir2.1, styrax, which is a kind of natural compound selected from traditional Chinese medicine. Our data show that styrax can abolish the inward and outward currents of Kir2.1. The IC 50 of styrax for WT, D172N and E299V are 0.0113 § 0.00075, 0.0204 § 0.0048 and 0.0122 § 0.0012 (in volume), respectively. The results indicate that styrax can serve as a novel blocker for Kir2.1.

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Section: Resultsmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Styrax blocks inward and outward current of Kir2.1 channel

Ren

Pang

et al. 2016

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“…If the QTc distance is measured as <360 msec for girls and as <350 msec for boys, it is diagnosed in the presence of one or more of the following conditions: pathogenic mutation, family history of SQTS, history of sudden death below the age of 40 years, and surviving a VT/VF episode without any cardiac diseases (3,4). During EPS, AERP and VERP are typically measured to be short.…”

Section: Discussionmentioning

confidence: 99%

“…An accelerated ventricular repolarization (VR) abnormality develops in the heart due to an electrical stability disorder secondary to increased extracellular potassium flow in the heart (2, 3). Therefore, ventricular arrhythmias develop, which cause syncope, convulsion, and SD (2).…”

Section: Introductionmentioning

confidence: 99%

Short QT syndrome in a 14-year-old patient: The first pediatric case from Turkey

Ergül

Özyılmaz²,

Onan³

et al. 2015

Anatol J Cardiol

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Short QT syndrome in pediatrics

et al. 2017

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Short QT syndrome is a malignant cardiac disease characterized by the presence of ventricular tachyarrhythmias leading to syncope and sudden cardiac death. Currently, international guidelines establish diagnostic criteria when QTc is below 340 ms. This entity is one of the main diseases responsible for sudden cardiac death in the pediatric population. In recent years, clinical, genetic and molecular advances in pathophysiological mechanisms related to short QT syndrome have improved diagnosis, risk stratification, and preventive measures. Despite these advances, automatic implantable cardiac defibrillator remains the most effective measure. Currently, six genes have been associated with short QT syndrome, which account for nearly 60% of clinically diagnosed families. Here, we review the main clinical hallmarks of the disease, focusing on the pediatric population.

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Short QT Syndrome: A Predictable Story

Cited by 20 publications

References 42 publications

Styrax blocks inward and outward current of Kir2.1 channel

Styrax blocks inward and outward current of Kir2.1 channel

Short QT syndrome in a 14-year-old patient: The first pediatric case from Turkey

Short QT syndrome in pediatrics

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