LQTS (long QT syndrome) is an important cause of cardiac sudden death. LQTS is characterized by a prolongation of the QT interval on an electrocardiogram. This prolongation predisposes the individual to torsade-de-pointes and subsequent sudden death by ventricular fibrillation. Mutations in a number of genes that encode ion channels have been implicated in LQTS. Hereditary mutations in the α- and β-subunits, KCNQ1 and KCNE1 respectively, of the K+ channel pore IKs are the commonest cause of LQTS and account for LQTS types 1 and 5 respectively (LQT1 and LQT5). Recently, it has been shown that disease pathogenesis in LQT1 can be influenced by the abnormal trafficking of KCNQ1. In comparison, whether defective trafficking of KCNE1 plays a role in LQT5 is less well established.
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November 2007
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Conference Article|
October 25 2007
The role of abnormal trafficking of KCNE1 in long QT syndrome 5
S.C. Harmer;
S.C. Harmer
1BHF Laboratories and Department of Medicine, University College London, 5 University Street, London WC1E 6JJ, U.K.
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A. Tinker
A. Tinker
1
1BHF Laboratories and Department of Medicine, University College London, 5 University Street, London WC1E 6JJ, U.K.
1To whom correspondence should be addressed (email a.tinker@ucl.ac.uk).
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Publisher: Portland Press Ltd
Received:
July 05 2007
Online ISSN: 1470-8752
Print ISSN: 0300-5127
© The Authors Journal compilation © 2007 Biochemical Society
2007
Biochem Soc Trans (2007) 35 (5): 1074–1076.
Article history
Received:
July 05 2007
Citation
S.C. Harmer, A. Tinker; The role of abnormal trafficking of KCNE1 in long QT syndrome 5. Biochem Soc Trans 1 November 2007; 35 (5): 1074–1076. doi: https://doi.org/10.1042/BST0351074
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