Pharmacological rescue of human K(+) channel long-QT2 mutations: human ether-a-go-go-related gene rescue without block.

@article{Rajamani2002PharmacologicalRO,
  title={Pharmacological rescue of human K(+) channel long-QT2 mutations: human ether-a-go-go-related gene rescue without block.},
  author={Sridharan Rajamani and Corey L. Anderson and Blake D. Anson and Craig T. January},
  journal={Circulation},
  year={2002},
  volume={105 24},
  pages={2830-5}
}
BACKGROUND Defective protein trafficking is a consequence of gene mutations. Human long-QT (LQT) syndrome results from mutations in several genes, including the human ether-a-go-go-related gene (HERG), which encodes a delayed rectifier K(+) current. Trafficking-defective mutant HERG protein is a mechanism for reduced delayed rectifier K(+) current in LQT2, and high-affinity HERG channel-blocking drugs can result in pharmacological rescue. Methods and Results- We postulated that drug molecules… CONTINUE READING

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