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J. Cardiovasc. Dev. Dis. 2017, 4(4), 16; doi:10.3390/jcdd4040016

Multiple Roles of Pitx2 in Cardiac Development and Disease

1
Cardiovascular Development Group, Department of Experimental Biology, University of Jaén, Jaén 23071, Spain
2
Institute of Anatomy, Charles University, and Institute of Physiology, Czech Academy of Sciences, Prague 128 00, Czech Republic
3
School of Biological Sciences, University of East Anglia, Norwich NR4 7TJ, UK
*
Author to whom correspondence should be addressed.
Received: 6 August 2017 / Revised: 2 October 2017 / Accepted: 3 October 2017 / Published: 11 October 2017
(This article belongs to the Special Issue Left–Right Asymmetry and Cardiac Morphogenesis)
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Abstract

Cardiac development is a complex morphogenetic process initiated as bilateral cardiogenic mesoderm is specified at both sides of the gastrulating embryo. Soon thereafter, these cardiogenic cells fuse at the embryonic midline configuring a symmetrical linear cardiac tube. Left/right bilateral asymmetry is first detected in the forming heart as the cardiac tube bends to the right, and subsequently, atrial and ventricular chambers develop. Molecular signals emanating from the node confer distinct left/right signalling pathways that ultimately lead to activation of the homeobox transcription factor Pitx2 in the left side of distinct embryonic organ anlagen, including the developing heart. Asymmetric expression of Pitx2 has therefore been reported during different cardiac developmental stages, and genetic deletion of Pitx2 provided evidence of key regulatory roles of this transcription factor during cardiogenesis and thus congenital heart diseases. More recently, impaired Pitx2 function has also been linked to arrhythmogenic processes, providing novel roles in the adult heart. In this manuscript, we provide a state-of-the-art review of the fundamental roles of Pitx2 during cardiogenesis, arrhythmogenesis and its contribution to congenital heart diseases. View Full-Text
Keywords: Pitx2; left/right signaling; congenital heart diseases; atrial fibrillation Pitx2; left/right signaling; congenital heart diseases; atrial fibrillation
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This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. (CC BY 4.0).

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Franco, D.; Sedmera, D.; Lozano-Velasco, E. Multiple Roles of Pitx2 in Cardiac Development and Disease. J. Cardiovasc. Dev. Dis. 2017, 4, 16.

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