El factor de transcripción Zfhx3 regula la expresión del gen SCN5A y la densidad de la corriente de sodio (INa) en el miocardio

Zusammenfassung

nherited arrhythmogenic syndromes (IAS) are rare diseases genetically and phenotypically complex that alter the electrical properties enhancing the arrhythmogenic risk in subjects with structurally normal hearts. Atrial fibrillation (AF) is a tachyarrhythmia characterized by an uncoordinated and very rapid activation of the atria. It is the most prevalent cardiac arrhythmia in the general population (1-2%). The risk of AF increases markedly with age and is more frequent in subjects with hypertension and various cardiovascular diseases, as well as in subjects with single nucelotide polymorphisms (SNPs) identified in population studies. Among the genes whose SNPs confer an increased risk of AF are those that encode several transcription factors (TF) including: Pitx2c, Tbx5, and Zfhx3. There are also inherited or familial forms of AF in which lone AF appears in young subjects following a mendelian hereditary pattern. Brugada syndrome (BrS) is a IAS characterized by a ST-segment elevation in the right precordial leads of the ECG and associated with an increased risk of ventricular fibrillation and sudden cardiac death (SCD)...