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KV7 channelopathies

  • Ion Channels, Receptors and Transporters
  • Published:
Pflügers Archiv - European Journal of Physiology Aims and scope Submit manuscript

Abstract

KV7 voltage-gated potassium channels, encoded by the KCNQ gene family, have caught increasing interest of the scientific community for their important physiological roles, which are emphasized by the fact that four of the five so far identified members are related to different hereditary diseases. Furthermore, these channels prove to be attractive pharmacological targets for treating diseases characterized by membrane hyperexcitability. KV7 channels are expressed in brain, heart, thyroid gland, pancreas, inner ear, muscle, stomach, and intestines. They give rise to functionally important potassium currents, reduction of which results in pathologies such as long QT syndrome, diabetes, neonatal epilepsy, neuromyotonia, or progressive deafness. Here, we summarize some key traits of KV7 channels and review how their molecular deficiencies could explain diverse disease phenotypes. We also assess the therapeutic potential of KV7 channels; in particular, how the activation of KV7 channels by the compounds retigabine and R-L3 may be useful for treatment of epilepsy or cardiac arrhythmia.

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Acknowledgements

This work was supported by the Federal Ministry for Education and Research (BMBF: E-rare/EuroBFNS/01GM0804 and NGFNplus/01GS08123), the European Union (Epicure: LSH 037315) and German Research Foundation (DFG SE1077/3-1).

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Authors and Affiliations

  1. Department of Neurology and Epileptology, Center for Neurology, Hertie Institute for Clinical Brain Research, University Hospital Tübingen, Hoppe-Seyler-Str. 3, 72076, Tübingen, Germany

    Snezana Maljevic & Holger Lerche

  2. MGH-HMS Center for Nervous System Repair, Departments of Neurosurgery and Neurology, Program in Neuroscience, Harvard Medical School, Boston, MA, USA

    Thomas V. Wuttke

  3. Department of Biochemistry I, Ruhr University Bochum, Bochum, Germany

    Guiscard Seebohm

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  1. Snezana Maljevic
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  2. Thomas V. Wuttke
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  3. Guiscard Seebohm
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  4. Holger Lerche
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Corresponding author

Correspondence to Holger Lerche.

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Snezana Maljevic, Thomas V. Wuttke, and Guiscard Seebohm contributed equally to this study.

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Maljevic, S., Wuttke, T.V., Seebohm, G. et al. KV7 channelopathies. Pflugers Arch - Eur J Physiol 460, 277–288 (2010). https://doi.org/10.1007/s00424-010-0831-3

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  • DOI: https://doi.org/10.1007/s00424-010-0831-3

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