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Clancy2001_Kchannel


ABSTRACT: 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. In summary, you are entitled to use this encoded model in absolutely any manner you deem suitable, verbatim, or with modification, alone or embedded it in a larger context, redistribute it, commercially or not, in a restricted way or not. 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.

DISEASE(S): Andersen-tawil Syndrome

SUBMITTER: Enuo He  

PROVIDER: BIOMD0000000121 | BioModels | 2024-09-02

REPOSITORIES: BioModels

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Publications

Cellular consequences of HERG mutations in the long QT syndrome: precursors to sudden cardiac death.

Clancy C E CE   Rudy Y Y  

Cardiovascular research 20010501 2


<h4>Background</h4>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.<h4>Objective</h4>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 EC  ...[more]

Publication: 1/5

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