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- Title
Axon initial segment dysfunction in epilepsy.
- Authors
Wimmer, Verena C.; Reid, Christopher A.; So, Eva Y.-W.; Berkovic, Samuel F.; Petrou, Steven
- Abstract
The axon initial segment (AIS) contains the site of action potential initiation and plays a major role in neuronal excitability. AIS function relies on high concentrations of different ion channels and complex regulatory mechanisms that orchestrate molecular microarchitecture. We review recent evidence that a large number of ion channels associated with epilepsy are enriched at the AIS, making it a ‘hotspot’ for epileptogenesis. Furthermore, we present novel data on the clustering of GABRγ2 receptors in the AIS of cortical and hippocampal neurons in a knock in mouse model of a human genetic epilepsy. This article highlights the molecular coincidence of epilepsy mutations at the AIS and reviews pathogenic mechanisms converging at the AIS.
- Publication
Journal of Physiology, 2010, Vol 588, Issue 11, p1829
- ISSN
0022-3751
- Publication type
Article
- DOI
10.1113/jphysiol.2010.188417