Molecular Pathophysiology of Congenital Long QT Syndrome.

  title={Molecular Pathophysiology of Congenital Long QT Syndrome.},
  author={Michael S. Bohnen and Gary Peng and Sharon Robey and C{\'e}cile Terrenoire and Vivek Iyer and Kevin J. Sampson and R. S. Kass},
  journal={Physiological reviews},
  volume={97 1},
Ion channels represent the molecular entities that give rise to the cardiac action potential, the fundamental cellular electrical event in the heart. The concerted function of these channels leads to normal cyclical excitation and resultant contraction of cardiac muscle. Research into cardiac ion channel regulation and mutations that underlie disease pathogenesis has greatly enhanced our knowledge of the causes and clinical management of cardiac arrhythmia. Here we review the molecular… CONTINUE READING