Document Detail

A defect in the Kv channel-interacting protein 2 (KChIP2) gene leads to a complete loss of I(to) and confers susceptibility to ventricular tachycardia.
MedLine Citation:
PMID:  11747815     Owner:  NLM     Status:  MEDLINE    
KChIP2, a gene encoding three auxiliary subunits of Kv4.2 and Kv4.3, is preferentially expressed in the adult heart, and its expression is downregulated in cardiac hypertrophy. Mice deficient for KChIP2 exhibit normal cardiac structure and function but display a prolonged elevation in the ST segment on the electrocardiogram. The KChIP2(-/-) mice are highly susceptible to the induction of cardiac arrhythmias. Single-cell analysis revealed a substrate for arrhythmogenesis, including a complete absence of transient outward potassium current, I(to), and a marked increase in action potential duration. These studies demonstrate that a defect in KChIP2 is sufficient to confer a marked genetic susceptibility to arrhythmias, establishing a novel genetic pathway for ventricular tachycardia via a loss of the transmural gradient of I(to).
H C Kuo; C F Cheng; R B Clark; J J Lin; J L Lin; M Hoshijima; V T Nguyêñ-Trân; Y Gu; Y Ikeda; P H Chu; J Ross; W R Giles; K R Chien
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  Cell     Volume:  107     ISSN:  0092-8674     ISO Abbreviation:  Cell     Publication Date:  2001 Dec 
Date Detail:
Created Date:  2001-12-18     Completed Date:  2002-01-17     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  0413066     Medline TA:  Cell     Country:  United States    
Other Details:
Languages:  eng     Pagination:  801-13     Citation Subset:  IM    
Institute of Molecular Medicine, UCSD-Salk Program in Molecular Medicine, School of Medicine, University of California, San Diego, La Jolla, CA 92093, USA.
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MeSH Terms
Action Potentials / physiology
Alternative Splicing
Amino Acid Sequence
Base Sequence
Calcium-Binding Proteins / chemistry,  genetics*,  metabolism
Embryo, Mammalian / metabolism
Gene Targeting
Genetic Predisposition to Disease*
In Situ Hybridization
Kv Channel-Interacting Proteins
Membrane Potentials / physiology
Mice, Knockout
Models, Biological
Molecular Sequence Data
Myocardium / cytology,  metabolism*
Patch-Clamp Techniques
Potassium / metabolism*
Potassium Channels / genetics,  metabolism
Potassium Channels, Voltage-Gated*
Protein Isoforms
Shal Potassium Channels
Tachycardia, Ventricular / etiology,  genetics*,  physiopathology
Reg. No./Substance:
0/Calcium-Binding Proteins; 0/KCND2 protein, human; 0/KCND3 protein, human; 0/KCNIP2 protein, human; 0/Kcnd2 protein, mouse; 0/Kcnd3 protein, mouse; 0/Kcnip2 protein, mouse; 0/Kv Channel-Interacting Proteins; 0/Potassium Channels; 0/Potassium Channels, Voltage-Gated; 0/Protein Isoforms; 0/Shal Potassium Channels; 7440-09-7/Potassium

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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