Brugada syndrome (BrS) has been named after the description of the disease made by the Brugada brothers in 1992. BrS is clinically characterized by arrhythmic events, in particular ventricular fibrillation, resulting in syncope and sudden cardiac arrest mainly in middle-aged men. The ECG shows a peculiar down-sloping elevation of the ST segment in the right pre-cordial ECG leads with inversion ...

The Hairy-related transcription-factor (HRT) genes encode three related basic helix-loop-helix transcription factors that show sequence similarity to the Hairy and Enhancer of split family of transcriptional repressors. HRT proteins are expressed in specific regions of the developing heart, vasculature, pharyngeal arches and somites, and the periodicity of their expression in somitic precursors...

Pulmonary hypertension (PH) is a fatal disease that lacks an effective therapy. Notch signaling pathway plays a crucial role in the angiogenesis and vascular remodeling. However, its roles in vascular remodeling in PH have not been well studied. In the current study, using hypoxia-induced PH model in rat, we examined the expression of Notch and its downstream factors. Then, we used vessel strip...

Urban traffic networks are inherently unstable when congested. This instability causes a natural tendency towards spatially inhomogeneous vehicle distributions and less consistent and reproducible relationships between urban traffic variables. It is important to find ways to mitigate this unstable behavior since well-defined relationships between average network flow and density—the MFD—are use...

Notch signaling regulates smooth muscle cell phenotype and is critical for vascular development. One Notch target is smooth muscle alpha-actin (SMA), a differentiated smooth muscle cell marker. The Notch intracellular domain (NotchICD) forms a complex with CBF-1 (C-promoter-binding factor-1) and directly induces SMA expression. Using primary human smooth muscle cells, we show that expression of...

Members of the Hairy/Enhancer of Split family of basic helix-loop-helix (bHLH) transcription factors are regulated by the Notch signaling pathway in vertebrate and Drosophila embryos and control cell fates and establishment of sharp boundaries of gene expression. Here, we describe a new subclass of bHLH proteins, HRT1 (Hairy-related transcription factor 1), HRT2, and HRT3, that share high homol...

1. Sotoodehnia N, et al. Common variants in 22 loci are associated with QRS duration and cardiac ventricular conduction. Nat Genet. 2010;42(12):1068–1076. 2. Holm H, et al. Several common variants modulate heart rate, PR interval and QRS duration. Nat Genet. 2010;42(2):117–122. 3. Pfeufer A, et al. Genome-wide association study of PR interval. Nat Genet. 2010;42(2):153–159. 4. Chambers JC, et a...

Ding et al. report in the current number of the AMS [1] a 12 year old patient with abdominal coarctation of the aorta above the renal arteries treated successfully with percutanous transluminal angioplasty. This report is a useful reminder that hypertension maybe the sole manifestation of aortic coarctation that has an estimated incidence of 1 in 2500 live births [2] and may vary from a single ...

Although congenital heart disease (CHD) is frequently associated with syndromes that affect multiple organs, the majority of cases present as isolated CHD, and typically defects are limited to a defined structure, suggesting a unique developmental mechanism. Despite the frequent occurrence of CHD, relatively few causative genes have been clearly identified (see review1). In this issue of Circul...

Artery Disease: Right Idea, Wrong Gene Variant? To the Editor: Recent data reported by Lin et al1 suggest that coronary artery disease (CAD) is associated with an Asn363Ser variant in exon 2 of the glucocorticoid receptor gene (GRL). The study is based on the notion that a dysfunctional glucocorticoid receptor may add to the adverse health effects of excessive cortisol concentrations. In a prev...