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J. Cardiovasc. Dev. Dis. 2014, 1(1), 52-72; doi:10.3390/jcdd1010052

Left Right Patterning, Evolution and Cardiac Development

Department of Cardiovascular Medicine and Wellcome Trust Centre for Human Genetics, University of Oxford, Roosevelt Drive, Headington, Oxford, OX3 7BN, UK
Received: 30 January 2014 / Revised: 24 March 2014 / Accepted: 28 March 2014 / Published: 8 April 2014
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Many aspects of heart development are determined by the left right axis and as a result several congenital diseases have their origins in aberrant left-right patterning. Establishment of this axis occurs early in embryogenesis before formation of the linear heart tube yet impacts upon much later morphogenetic events. In this review I discuss the differing mechanisms by which left-right polarity is achieved in the mouse and chick embryos and comment on the evolution of this system. I then discus three major classes of cardiovascular defect associated with aberrant left-right patterning seen in mouse mutants and human disease. I describe phenotypes associated with the determination of atrial identity and venous connections, looping morphogenesis of the heart tube and finally the asymmetric remodelling of the embryonic branchial arch arterial system to form the leftward looped arch of aorta and associated great arteries. Where appropriate, I consider left right patterning defects from an evolutionary perspective, demonstrating how developmental processes have been modified in species over time and illustrating how comparative embryology can aide in our understanding of congenital heart disease.
Keywords: left-right patterning; heterotaxy; isomerism; Nodal; Pitx2c left-right patterning; heterotaxy; isomerism; Nodal; Pitx2c
This is an open access article distributed under the Creative Commons Attribution License (CC BY 3.0).

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Dykes, I.M. Left Right Patterning, Evolution and Cardiac Development. J. Cardiovasc. Dev. Dis. 2014, 1, 52-72.

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J. Cardiovasc. Dev. Dis. EISSN 2308-3425 Published by MDPI AG, Basel, Switzerland RSS E-Mail Table of Contents Alert
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