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J Physiol. 2010 Jun 1;588(Pt 11):1829-40. doi: 10.1113/jphysiol.2010.188417. Epub 2010 Apr 7.

Axon initial segment dysfunction in epilepsy.

Author information

1
Florey Neuroscience Institutes, University of Melbourne, Parkville 3010, Victoria, Australia.

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 GABRgamma2 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.

PMID:
20375142
PMCID:
PMC2901971
DOI:
10.1113/jphysiol.2010.188417
[Indexed for MEDLINE]
Free PMC Article

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