Consequences of HERG mutations in the long QT syndrome (Clancy, Rudy 2001)

This study demonstrates which mutations can prolong APD sufficiently to generate early afterdepolarizations (EADs), which may trigger life-threatening arrhythmias. The severity of the phenotype is shown to depend on the specific kinetic changes and how they affect I(Kr) during the time course of the action potential. See paper for more and details.

Model Type: Channel/Receptor

Cell Type(s): Heart cell

Currents: I K; I_HERG

Genes: HERG KCNH2

Model Concept(s): Ion Channel Kinetics; Heart disease; Long-QT

Simulation Environment: XPPAUT

Implementer(s): Wu, Sheng-Nan [snwu at mail.ncku.edu.tw]; Chang, Han-Dong; Wu, Jiun-Shian [coolneon at gmail.com]

References:

Clancy CE, Rudy Y. (2001). Cellular consequences of HERG mutations in the long QT syndrome: precursors to sudden cardiac death. Cardiovascular research. 50 [PubMed]