Disopyramide is an effective inhibitor of mutant HERG K+ channels involved in variant 1 short QT syndrome.

@article{McPate2006DisopyramideIA,
  title={Disopyramide is an effective inhibitor of mutant HERG K+ channels involved in variant 1 short QT syndrome.},
  author={Mark J. McPate and R. Scot Duncan and Harry J. Witchel and Jules C. Hancox},
  journal={Journal of molecular and cellular cardiology},
  year={2006},
  volume={41 3},
  pages={563-6}
}
The recently identified idiopathic short QT syndrome (SQTS) is associated with an increased risk of arrhythmia and sudden death. The use of implantable cardioverter defibrillators helps to protect SQTS patients from ventricular fibrillation; however, pharmacological treatments to normalise the QT interval are limited: thus far only quinidine has been found to be effective in a subset of patients, with the SQT1 variant. SQT1 is associated with an amino acid substitution (N588K) in the KCNH2… CONTINUE READING

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