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(Circulation Research. 2007;100:764.)
© 2007 American Heart Association, Inc.

Editorials

Hey, There’s a Hole in My Heart

Stephanie A. Stowell, Jan Kitajewski

From the Departments of Pathology (S.A.S., J.K.) and Ob/Gyn (J.K.), Columbia University Medical Center, New York, NY.

Correspondence to Jan Kitajewski, PhD, Irving Cancer Research Center, 1130 St. Nicholas Avenue, Columbia University, New York, NY 10032. E-mail jkk9@columbia.edu

See related article, pages 856–863

Key Words: Notch • Hey • heart • septal defects • epithelial mesenchymal transition

An extract of the first 250 words of the full text is provided, because this article has no abstract.

Mutations in genes encoding the developmental regulators Notch cause an amalgam of defects such that the many roles of this signaling receptor cannot be teased apart by studying the global loss of Notch alone. Instead more subtle and elegant genetic manipulations are called for to discern the multiple roles of Notch. In the current issue of Circulation Research Fischer et al describe a major step toward the understanding of Notch signaling not through the manipulation of the receptor itself, but by studying the loss of two of its downstream effectors.¹ Alone, neither the loss of function of Hey1 nor HeyL appear as necessary contributors to proper heart development. Loss of both Hey1 and HeyL however, leads to atrioventricular (AV) dysplasia and membranous ventricular septal defects during cardiogenesis. These phenotypes mimic common congenital heart defects found in the human population and demonstrate a more accurate mouse model that can be used in studying human disease and genetic anomalies.

Despite medical advances, congenital heart disease among children and infants leading to heart failure is still a common occurrence. In fact congenital heart disease is the most common birth defect among live births with an incidence of roughly 1%.² In these patients, ventricular septal defects and atrial septal defects are the most frequent forms of congenital heart defects found. Loss of separation between the left and right sides of the heart results in the inefficient recirculation of oxygenated blood. The over-burdened heart becomes enlarged and sets the stage for overall heart failure. In . . . [Full Text of this Article]

Related Article:

Combined Loss of Hey1 and HeyL Causes Congenital Heart Defects Because of Impaired Epithelial to Mesenchymal Transition

Andreas Fischer, Christian Steidl, Toni U. Wagner, Esra Lang, Peter M. Jakob, Peter Friedl, Klaus-Peter Knobeloch, and Manfred Gessler
Circ. Res. 2007 100: 856-863. [Abstract] [Full Text] [PDF]

This article has been cited by other articles:

				K. A. Sharff, W.-X. Song, X. Luo, N. Tang, J. Luo, J. Chen, Y. Bi, B.-C. He, J. Huang, X. Li, et al. Hey1 Basic Helix-Loop-Helix Protein Plays an Important Role in Mediating BMP9-induced Osteogenic Differentiation of Mesenchymal Progenitor Cells J. Biol. Chem., January 2, 2009; 284(1): 649 - 659. [Abstract] [Full Text] [PDF]