The Brugada Syndrome Susceptibility Gene HEY2 Modulates Cardiac Transmural Ion Channel Patterning and Electrical Heterogeneity

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Letter by El-Battrawy et al Regarding Article, "The Brugada Syndrome Susceptibility Gene HEY2 Modulates Cardiac Transmural Ion Channel Patterning and Electrical Heterogeneity".

RATIONALE Genome-wide association studies previously identified an association of rs9388451 at chromosome 6q22.3 (near HEY2) with Brugada syndrome. The causal gene and underlying mechanism remain unresolved. OBJECTIVE We used an integrative approach entailing transcriptomic studies in human hearts and electrophysiological studies in Hey2+/- (Hey2 heterozygous knockout) mice to dissect the und...

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A New Susceptibility Gene for Brugada Syndrome

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Electrical heterogeneity, cardiac arrhythmias, and the sodium channel.

It was not long ago that we thought of the ventricles of the heart as being composed of 2 basic cell types: specialized conducting cells that make up the His-Purkinje system and ventricular myocytes. Studies conducted over the last decade have highlighted the diversity among the cells that comprise the ventricular myocardium, pointing to regional differences in the electrical properties of cell...

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MOG1: a new susceptibility gene for Brugada syndrome.

BACKGROUND Brugada syndrome (BrS) is caused mainly by mutations in the SCN5A gene, which encodes the α-subunit of the cardiac sodium channel Na(v)1.5. However, ≈ 20% of probands have SCN5A mutations, suggesting the implication of other genes. MOG1 recently was described as a new partner of Na(v)1.5, playing a potential role in the regulation of its expression and trafficking. We investigated wh...

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Brugada syndrome and neurally mediated susceptibility.

The risk of sudden death in patients with Brugada syndrome (BS) is still unclear. Moreover, particular clinical conditions may have a confounding effect on the diagnostic and therapeutic approach. We report the case of a 27-year-old man with a clinical history of suspected neurally mediated syncope and typical ECG features of BS. The tilt table test showed a type I, mixed, positive response. Th...

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ژورنال

عنوان ژورنال: Circulation Research

سال: 2017

ISSN: 0009-7330,1524-4571

DOI: 10.1161/circresaha.117.310959