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Cardiac channelopathies

Nature volume 415, pages 213–218 (2002)Cite this article

Abstract

Genetic alterations of various ion channels produce heritable cardiac arrhythmias that predispose affected individuals to sudden death. The investigation of such 'channelopathies' continues to yield remarkable insights into the molecular basis of cardiac excitability. The concept of channelopathies is not restricted to genetic disorders; notably, changes in the expression or post-translational modification of ion channels underlie the fatal arrhythmias associated with heart failure. Recognizing the fundamental defects in channelopathies provides the basis for new strategies of treatment, including tailored pharmacotherapy and gene therapy.

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Figure 1: Ion channels underlie cardiac excitability.
Figure 2: The action potential and the electrocardiogram (ECG).
Figure 3: Explanation of the mechanism underlying arrhythmia in long-QT syndrome.
Figure 4: Computational rationalization of heart failure arrhythmias on the basis of known cellular changes in ionic currents.

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Acknowledgements

Supported by NIH. E.M. is the Michel Mirowski, M.D. Professor of Cardiology of the Johns Hopkins University.

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  1. Institute of Molecular Cardiobiology, Ross 844, The Johns Hopkins University, Baltimore, 21205, Maryland, USA

    Eduardo Marbán

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  1. Eduardo Marbán
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Correspondence to Eduardo Marbán.

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Marbán, E. Cardiac channelopathies. Nature 415, 213–218 (2002). https://doi.org/10.1038/415213a

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