SP4 and atrioventricular block: Mef2c and Klf13 are reported functional interaction partners of Tbx5 in the regulation of cardiac development and gene regulation.25,26 The Sp4 TF is expressed in the heart, including the atria and AV node, and Sp4-deficient mice display a high incidence of sinus bradycardia and AV block.50 Future studies could explore whether the Tbx5-p.G125R interactions with Mef2c—as well as the Sp and Klf TF family members expressed in the atria—are affected.