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(Circulation Research. 2008;102:1304.)
© 2008 American Heart Association, Inc.

Editorials

Holt–Oram Syndrome and Atrial Fibrillation

Opening the (T)-Box

Elisabetta Cerbai, Laura Sartiani

From the Center of Molecular Medicine (C.I.M.M.B.A.), University of Florence, Italy.

Correspondence to Elisabetta Cerbai, PhD, Center of Molecular Medicine, University of Florence, Viale G. Pieraccini 6, 50139 Firenze, Italy. E-mail elisabetta.cerbai@unifi.it

See related article, pages 1433–1442

Key Words: T-box genes • cardiac development • atrial fibrillation • Holt-Oram Syndrome

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

	Introduction

 
Cardiogenesis is a complex phenomenon: its success—and ultimately life births—depends on factors acting in a combinatorial or hierarchical fashion and turning on and off gene transcription. Actually, the incidence of cardiac defects at birth is relatively high (1% to 2%), and our comprehension of these phenomena very limited. Hence, the role of transcription factors in cardiac specification and maturation has claimed increasing attention in recent years, providing a complex and evolving picture of the molecular and cellular processes involved but leaving many questions unanswered. In this issue of Circulation Research, Postma et al¹ add to a growing list of regulatory factors/functions a step forward in our understanding of cardiac morphogenesis and disclosing novel features of Tbx5.

	T-Box Genes and Heart Development

 
Tbx5 belongs to the T-box gene family; the first member was identified in 1927 by a genetist who selected a mouse strain with truncated tail carrying a heterozygous mutation in a locus called T (reviewed in²). More than 60 years later the gene was cloned and named Brachyury (short-tail in Greek),³ but its functional role remained obscure because the T-gene product lacked homology to any previously characterized protein. Between 1993 and 1997, it was described as a novel DNA-binding protein and the crystallographic structure of the domain, termed the T-box, revealed a new feature of protein-DNA interaction.⁴ In recent years, studies in transgenic mice lines and in patients carrying spontaneous mutations have demonstrated that T-box genes act as crucial regulators for the morphogenesis of a wide range of tissues and . . . [Full Text of this Article]

Related Article:

A Gain-of-Function TBX5 Mutation Is Associated With Atypical Holt–Oram Syndrome and Paroxysmal Atrial Fibrillation

Alex V. Postma, Judith B.A. van de Meerakker, Inge B. Mathijssen, Phil Barnett, Vincent M. Christoffels, Aho Ilgun, Jan Lam, Arthur A.M. Wilde, Ronald H. Lekanne Deprez, and Antoon F.M. Moorman
Circ. Res. 2008 102: 1433-1442. [Abstract] [Full Text] [PDF]

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