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Trends Cardiovasc Med. 2014 Jan;24(1):23-31. doi: 10.1016/j.tcm.2013.06.001. Epub 2013 Aug 15.

Homeobox transcription factor Pitx2: The rise of an asymmetry gene in cardiogenesis and arrhythmogenesis.

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Department of Experimental Biology B3-362, University of Jaén, Jaen 23071, Spain. Electronic address:
Heart Failure Research Centre, Academic Medical Centre, Amsterdam, The Netherlands.
CNR-Institute of Neurosciences, Department of Biomedical Sciences, University of Padua, Padua, Italy.


The homeobox transcription factor Pitx2 displays a highly specific expression pattern during embryogenesis. Gain and loss of function experiments have unraveled its pivotal role in left-right signaling. Conditional deletion in mice has demonstrated a complex and intricate role for Pitx2 in distinct aspects of cardiac development and more recently a link to atrial fibrillation has been proposed based on genome-wide association studies. In this review we will revise the role of Pitx2 in the developing heart, starting from the early events of left-right determination followed by its role in cardiac morphogenesis and ending with its role in cardiac arrhythmogenesis.

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