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Mutations in  hERG  cause long QT syndrome type 2 which is characterized by a prolonged QT interval on electrocardiogram and predisposition to life-threatening ventricular tachyarrhythmia, syncope, and sudden death.  hERG-G572S  induces trafficking defects of  hERG  channel protein from Golgi to the plasma membrane and results in a dominant negative suppression of  hERG  current density. As an accessory β subunit,  KCNE2  promotes  hERG  migration from Golgi to cellular membrane. In this study, we investigated the rescue effect of  KCNE2  in a  G572S  mutation of  hERG . Transfection was performed into HEK293 cells. Patch clamp technique, western blotting analyses and confocal microscopic examination were used. Results showed that  KCNE2  had a significantly enhanced effect on  G572S  mutation current. The increase of current was largest at  KCNH2 : KCNE2  of 1:3. Confocal images showed co-expressing  G572S  and  KCNE2  could cause a substantial up-regulated membrane protein (155 kDa) expression. Expression of membrane protein accumulated markedly with increasing ratio of  KCNH2 : KCNE2 .  G572S  defective mutant could be restored by both  KCNE2  and lower temperature (27°C), which suggested that the lower temperature could be the favorable circumstances for the rescue function of  KCNE2 . In this study, we successfully set up “the action potential” on the HEK 293 cells by genetically engineered to express Kir2.1, Nav1.5, and Kv11.1, wherein on reaching over an excitation threshold by current injection. The results suggested that  KCNE2  could shorten action potential duration which was prolonged by  G572S . These findings described electrophysiological characteristics of the LQT2 syndrome mutation  KCNH2-G572S  and regulation by accessory protein  KCNE2 , and provided a clue about LQT2 and relative rescue mechanism.

Electrophysiological Characteristics of the LQT2 Syndrome Mutation KCNH2-G572S and Regulation by Accessory Protein KCNE2