Identification of a targeted and testable antiarrhythmic therapy for long-QT syndrome type 2 using a patient-specific cellular model.

@article{Mehta2018IdentificationOA,
  title={Identification of a targeted and testable antiarrhythmic therapy for long-QT syndrome type 2 using a patient-specific cellular model.},
  author={Ashish Mehta and Chrishan J. A. Ramachandra and Pritpal Singh and Anuja A Chitre and Chong Hui Lua and Manuela Mura and Lia Crotti and Philip E H Wong and Peter J. Schwartz and Massimiliano Gnecchi and Winston Shim},
  journal={European heart journal},
  year={2018},
  volume={39 16},
  pages={
          1446-1455
        }
}
Aims Loss-of-function mutations in the hERG gene causes long-QT syndrome type 2 (LQT2), a condition associated with reduced IKr current. Four different mutation classes define the molecular mechanisms impairing hERG. Among them, Class 2 mutations determine hERG trafficking defects. Lumacaftor (LUM) is a drug acting on channel trafficking already successfully tested for cystic fibrosis and its safety profile is well known. We hypothesize that LUM might rescue also hERG trafficking defects in… CONTINUE READING
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