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Reduced sodium current in GABAergic interneurons in a mouse model of severe myoclonic epilepsy in infancy

Nature neuroscience, 2006-09, Vol.9 (9), p.1142-1149 [Peer Reviewed Journal]

COPYRIGHT 2006 Nature Publishing Group ;Copyright Nature Publishing Group Sep 2006 ;ISSN: 1097-6256 ;EISSN: 1546-1726 ;DOI: 10.1038/nn1754 ;PMID: 16921370 ;CODEN: NANEFN

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Title:
Reduced sodium current in GABAergic interneurons in a mouse model of severe myoclonic epilepsy in infancy
Author: Catterall, William A ; Yu, Frank H ; Mantegazza, Massimo ; Westenbroek, Ruth E ; Robbins, Carol A ; Kalume, Franck ; Burton, Kimberly A ; Spain, William J ; McKnight, G Stanley ; Scheuer, Todd
Subjects: Action Potentials - physiology ; Analysis ; Animals ; Cell Line ; Disease Models, Animal ; Electroencephalography ; Epilepsies, Myoclonic - genetics ; Epilepsies, Myoclonic - physiopathology ; gamma-Aminobutyric Acid - metabolism ; Gene mutations ; Genetic aspects ; Genotype ; Humans ; Immunoblotting ; Infant ; Interneurons - cytology ; Interneurons - metabolism ; Interneurons - physiology ; Mice ; Mice, Inbred C57BL ; Mice, Inbred Strains ; Mice, Knockout ; Mutation - genetics ; Myoclonic epilepsy ; NAV1.1 Voltage-Gated Sodium Channel ; Nerve Tissue Proteins - genetics ; Nerve Tissue Proteins - metabolism ; Nerve Tissue Proteins - physiology ; Neural transmission ; Patch-Clamp Techniques ; Phenotype ; Regulation ; Seizures - genetics ; Seizures - mortality ; Seizures - physiopathology ; Sodium channels ; Sodium Channels - genetics ; Sodium Channels - metabolism ; Sodium Channels - physiology ; Survival Rate
Is Part Of: Nature neuroscience, 2006-09, Vol.9 (9), p.1142-1149
Description: Voltage-gated sodium channels (Na(V)) are critical for initiation of action potentials. Heterozygous loss-of-function mutations in Na(V)1.1 channels cause severe myoclonic epilepsy in infancy (SMEI). Homozygous null Scn1a-/- mice developed ataxia and died on postnatal day (P) 15 but could be sustained to P17.5 with manual feeding. Heterozygous Scn1a+/- mice had spontaneous seizures and sporadic deaths beginning after P21, with a notable dependence on genetic background. Loss of Na(V)1.1 did not change voltage-dependent activation or inactivation of sodium channels in hippocampal neurons. The sodium current density was, however, substantially reduced in inhibitory interneurons of Scn1a+/- and Scn1a-/- mice but not in their excitatory pyramidal neurons. An immunocytochemical survey also showed a specific upregulation of Na(V)1.3 channels in a subset of hippocampal interneurons. Our results indicate that reduced sodium currents in GABAergic inhibitory interneurons in Scn1a+/- heterozygotes may cause the hyperexcitability that leads to epilepsy in patients with SMEI.
Publisher: United States: Nature Publishing Group
Language: English
Identifier: ISSN: 1097-6256
EISSN: 1546-1726
DOI: 10.1038/nn1754
PMID: 16921370
CODEN: NANEFN
Source: ProQuest One Psychology
MEDLINE
ProQuest Central

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Reduced sodium current in GABAergic interneurons in a mouse model of severe myoclonic epilepsy in infancy

COPYRIGHT 2006 Nature Publishing Group ;Copyright Nature Publishing Group Sep 2006 ;ISSN: 1097-6256 ;EISSN: 1546-1726 ;DOI: 10.1038/nn1754 ;PMID: 16921370 ;CODEN: NANEFN

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