Reduced current density, partially rescued by mexiletine, and depolarizing shift in activation of SCN5A W374G channels as a cause of severe form of Brugada syndrome.
Tadashi NakajimaTommy DharmawanReika Kawabata-IwakawaShuntaro TamuraHiroshi HasegawaTakashi KobariMasaki OtaShoichi TangeMasahiko NishiyamaYoshiaki KanekoMasahiko KurabayashiPublished in: Annals of noninvasive electrocardiology : the official journal of the International Society for Holter and Noninvasive Electrocardiology, Inc (2021)
Reduced current density, possibly due to a trafficking defect, and depolarizing shift in activation of SCN5A W374G are underlying biophysical defects in this severe form of BrS. Trafficking defects of SCN5A mutations at D1 pore may be commonly rescued by MEX.
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