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The transient outward potassium current (I to ) plays a prominent role in the development of cardiac arrhythmias associated with the J-wave syndromes, including Brugada and early repolarization syndromes. The J-wave syndromes are strongly male-dominated, presumably due to higher expression of I to in the right ventricular epicardium of male vs female dogs. In this study we tested the...
Mutations in genes encoding the α (KCND3) and β (KCNE3) subunits of genes encoding the cardiac transient outward potassium (I to ) have been associated with inherited cardiac arrhythmia syndromes such as Brugada syndrome (BrS) and sudden infant death syndromes (SIDS). Here, we identify and characterize a novel genetic variant in DPP10 associated with severe pediatric J-wave syndrome (JWS)...
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