public model
Short description

This model is according to the paper Cellular consequences of HEGR mutations in the long QT syndrome: precursors to sudden cardiac death. The author used Markovian model of cardiac Ikr in the paper. Figure4B in the paper has been reproduced using CellDesigner3.5.1. The cell is depolarized to the indicated test potential for 250ms (from 50ms to 300ms) from a holding potential of -40mV and then repolarized to -40mV. Change the value for vtest from -30,-20,-10,0,10,20,30,40 for each simulation in order to produce the different cureve in the paper.

To the extent possible under law, all copyright and related or neighbouring rights to this encoded model have been dedicated to the public domain worldwide. Please refer to CC0 Public Domain Dedication for more information.

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To cite BioModels Database, please use: Li C, Donizelli M, Rodriguez N, Dharuri H, Endler L, Chelliah V, Li L, He E, Henry A, Stefan MI, Snoep JL, Hucka M, Le Novère N, Laibe C (2010) BioModels Database: An enhanced, curated and annotated resource for published quantitative kinetic models. BMC Syst Biol., 4:92.

Related Publication
  • Cellular consequences of HERG mutations in the long QT syndrome: precursors to sudden cardiac death.
  • Clancy CE, Rudy Y
  • Cardiovascular research , 5/ 2001 , Volume 50 , pages: 301-313
  • Cardiac Bioelectricity Research and Training Center, Department of Physiology and Biophysics, 509 Wickenden Building, Case Western Reserve University, Cleveland, Ohio 44106-7207, USA.
  • A variety of mutations in HERG, the major subunit of the rapidly activating component of the cardiac delayed rectifier I(Kr), have been found to underlie the congenital Long-QT syndrome, LQT2. LQT2 may give rise to severe arrhythmogenic phenotypes leading to sudden cardiac death.We attempt to elucidate the mechanisms by which heterogeneous LQT2 genotypes can lead to prolongation of the action potential duration (APD) and consequently the QT interval on the ECG.We develop Markovian models of wild-type (WT) and mutant I(Kr) channels and incorporate these models into a comprehensive model of the cardiac ventricular cell.Using this virtual transgenic cell model, we describe the effects of HERG mutations on the cardiac ventricular action potential (AP) and provide insight into the mechanism by which each defect results in a net loss of repolarizing current and prolongation of APD.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. Clarifying how defects in HERG can lead to impaired cellular electrophysiology can improve our understanding of the link between channel structure and cellular function.
Enuo He

Metadata information

BioModels Database MODEL2898180293
BioModels Database BIOMD0000000121
PubMed 11334834
PubMed 10448858
PubMed 7514509
PubMed 7788872
PubMed 10318671
Taxonomy Cavia porcellus
Mathematical Modelling Ontology Ordinary differential equation model
OMIM 152427
Human Disease Ontology Andersen-Tawil syndrome
Curation status
Original model(s)
Name Description Size Actions

Model files

BIOMD0000000121_url.xml SBML L2V1 representation of Clancy2001_Kchannel 24.98 KB Preview | Download

Additional files

BIOMD0000000121.m Auto-generated Octave file 6.24 KB Preview | Download
BIOMD0000000121_urn.xml Auto-generated SBML file with URNs 24.61 KB Preview | Download
BIOMD0000000121.png Auto-generated Reaction graph (PNG) 17.28 KB Preview | Download
BIOMD0000000121.vcml Auto-generated VCML file 28.45 KB Preview | Download
BIOMD0000000121.xpp Auto-generated XPP file 4.10 KB Preview | Download
BIOMD0000000121.svg Auto-generated Reaction graph (SVG) 11.14 KB Preview | Download
BIOMD0000000121-biopax3.owl Auto-generated BioPAX (Level 3) 18.11 KB Preview | Download
BIOMD0000000121.pdf Auto-generated PDF file 169.33 KB Preview | Download
BIOMD0000000121-biopax2.owl Auto-generated BioPAX (Level 2) 13.42 KB Preview | Download
BIOMD0000000121.sci Auto-generated Scilab file 67.00 bytes Preview | Download

  • Model originally submitted by : Enuo He
  • Submitted: Jul 5, 2007 9:14:39 AM
  • Last Modified: Apr 27, 2017 7:37:54 PM
  • Version: 2 public model Download this version
    • Submitted on: Apr 27, 2017 7:37:54 PM
    • Submitted by: Enuo He
    • With comment: Current version of Clancy2001_Kchannel
  • Version: 1 public model Download this version
    • Submitted on: Jul 5, 2007 9:14:39 AM
    • Submitted by: Enuo He
    • With comment: Original import of Clancy2001_Kchannel
Curator's comment:
(added: 26 Jun 2007, 23:52:30, updated: 26 Jun 2007, 23:52:30)
Figure4B has been reproduced by Celldesigner 3.5.1