Alterations of Nedd4-2-binding capacity in PY-motif of NaV 1.5 channel underlie long QT syndrome and Brugada syndrome.
Ya WangYuan DuLing LuoPeijing HuGuodong YangTao LiXiu HanAiqun MaTingzhong WangPublished in: Acta physiologica (Oxford, England) (2020)
Our data suggest that the PY-motif plays an essential role in modifying the expression/function of NaV 1.5 channels through Nedd4-2-mediated ubiquitination. Alterations of NaV 1.5-Nedd4-2 interaction represent a novel pathological mechanism for NaV 1.5 channel diseases caused by SCN5A variants.