Two novel Brugada syndrome-associated mutations increase KV4.3 membrane expression and function

@inproceedings{You2015TwoNB,
  title={Two novel Brugada syndrome-associated mutations increase KV4.3 membrane expression and function},
  author={Tao You and Weike Mao and Benzhi Cai and Faqian Li and Haodong Xu},
  booktitle={International journal of molecular medicine},
  year={2015}
}
The human cardiac fast transient outward K+ channel is composed of the KV4.3 α subunit encoded by KCND3 and the K+ channel‑interacting protein 2 (KChIP2) β subunit, and determines the early repolarization of the action potential (AP). Two human mutations (G600R and L450F) in KV4.3 are associated with Brugada syndrome and they increase the KV4.3/KChIP2‑encoded fast transient outward K+ current (Ito,f) and cause the stable loss of the AP dome. However, the detailed mechanisms underlying the gain… CONTINUE READING