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Schematic illustration of PITX2 signaling in atria. Lefty1 represses PITX2 expression in the right atrium (RA), resulting in upregulation of Shox2 that induces sinoatrial nodal (SAN) gene expression through Tbx3. In the left atrium (LA) and pulmonary vein (PV) myocardium, PITX2 is upregulated, leading to downregulation of Shox2 and upregulation of Nkx2.5, which mediates the working myocardium gene expression [31].
