Potassium Channelopathies of Epilepsy

Robert Brenner and Karen S. Wilcox

in Jasper's Basic Mechanisms of the Epilepsies

Fourth edition

Published on behalf of ©Jeffrey L. Noebels, Massimo Avoli, Michael A. Rogawski, Richard W. Olsen, and Antonio V. Delgado-Escueta

Published in print July 2012 | ISBN: 9780199746545
Published online April 2013 | e-ISBN: 9780199322817 | DOI:

Series: Contemporary Neurology Series

Potassium Channelopathies of Epilepsy

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An exceedingly large number of familial and de novo channelopathies in several different types of potassium channels have already been found to underlie, or be associated with, many types of epilepsy. Given that the role of most potassium channels is to contribute to the maintenance of membrane hyperpolarization and repolarization, it is not surprising that loss-of-function mutations contribute to epilepsy. However, recently described potassium channelopathies resulting in gain of function can also, paradoxically, result in epilepsy. Furthermore, as many LQTS mutations arise in potassium channels, a link between epilepsy, SUDEP, and LQTS, as has now been observed for KCNQ1, may begin to inform prevention strategies for patients at risk for SUDEP. Finally, animal models harboring human mutations found in potassium channels have contributed greatly to our understanding of the mechanisms whereby specific channelopathies contribute to epilepsy, and it is anticipated that as this field continues to develop, advances in treatment strategies for patients will also be elucidated from such animal models.

Chapter.  8933 words.  Illustrated.

Subjects: Neurology

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