Deletion of the K(v)1.1 Potassium channel causes epilepsy in mice

Sharon L. Smart, Valeri Lopantsev, C. L. Zhang, Carol A. Robbins, Hao Wang, S. Y. Chiu, Philip A Schwartzkroin, Albee Messing, Bruce L. Tempel

Research output: Contribution to journalArticle

412 Citations (Scopus)

Abstract

Mice lacking the voltage-gated potassium channel α subunit, K(v)1.1, display frequent spontaneous seizures throughout adult life. In hippocampal slices from homozygous K(v)1.1 null animals, intrinsic passive properties of CA3 pyramidal cells are normal. However, antidromic action potentials are recruited at lower thresholds in K(v)1.1 null slices. Furthermore, in a subset of slices, mossy fiber stimulation triggers synaptically mediated long-latency epileptiform burst discharges. These data indicate that loss of K(v)1.1 from its normal localization in axons and terminals of the CA3 region results in increased excitability in the CA3 recurrent axon collateral system, perhaps contributing to the limbic and tonic-clonic components of the observed epileptic phenotype. Axonal action potential conduction was altered as well in the sciatic nerve-a deficit potentially related to the pathophysiology of episodic ataxia/myokymia, a disease associated with missense mutations of the human K(v)1.1 gene.

Original languageEnglish (US)
Pages (from-to)809-819
Number of pages11
JournalNeuron
Volume20
Issue number4
DOIs
StatePublished - Apr 1998
Externally publishedYes

Fingerprint

Potassium Channels
Action Potentials
Epilepsy
Myokymia
Voltage-Gated Potassium Channels
Pyramidal Cells
Presynaptic Terminals
Sciatic Nerve
Missense Mutation
Axons
Seizures
Phenotype
Genes
Episodic Ataxia

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Smart, S. L., Lopantsev, V., Zhang, C. L., Robbins, C. A., Wang, H., Chiu, S. Y., ... Tempel, B. L. (1998). Deletion of the K(v)1.1 Potassium channel causes epilepsy in mice. Neuron, 20(4), 809-819. https://doi.org/10.1016/S0896-6273(00)81018-1

Deletion of the K(v)1.1 Potassium channel causes epilepsy in mice. / Smart, Sharon L.; Lopantsev, Valeri; Zhang, C. L.; Robbins, Carol A.; Wang, Hao; Chiu, S. Y.; Schwartzkroin, Philip A; Messing, Albee; Tempel, Bruce L.

In: Neuron, Vol. 20, No. 4, 04.1998, p. 809-819.

Research output: Contribution to journalArticle

Smart, SL, Lopantsev, V, Zhang, CL, Robbins, CA, Wang, H, Chiu, SY, Schwartzkroin, PA, Messing, A & Tempel, BL 1998, 'Deletion of the K(v)1.1 Potassium channel causes epilepsy in mice', Neuron, vol. 20, no. 4, pp. 809-819. https://doi.org/10.1016/S0896-6273(00)81018-1
Smart SL, Lopantsev V, Zhang CL, Robbins CA, Wang H, Chiu SY et al. Deletion of the K(v)1.1 Potassium channel causes epilepsy in mice. Neuron. 1998 Apr;20(4):809-819. https://doi.org/10.1016/S0896-6273(00)81018-1
Smart, Sharon L. ; Lopantsev, Valeri ; Zhang, C. L. ; Robbins, Carol A. ; Wang, Hao ; Chiu, S. Y. ; Schwartzkroin, Philip A ; Messing, Albee ; Tempel, Bruce L. / Deletion of the K(v)1.1 Potassium channel causes epilepsy in mice. In: Neuron. 1998 ; Vol. 20, No. 4. pp. 809-819.
@article{6a7e815b9cc742b49ca637a87fa3fe9b,
title = "Deletion of the K(v)1.1 Potassium channel causes epilepsy in mice",
abstract = "Mice lacking the voltage-gated potassium channel α subunit, K(v)1.1, display frequent spontaneous seizures throughout adult life. In hippocampal slices from homozygous K(v)1.1 null animals, intrinsic passive properties of CA3 pyramidal cells are normal. However, antidromic action potentials are recruited at lower thresholds in K(v)1.1 null slices. Furthermore, in a subset of slices, mossy fiber stimulation triggers synaptically mediated long-latency epileptiform burst discharges. These data indicate that loss of K(v)1.1 from its normal localization in axons and terminals of the CA3 region results in increased excitability in the CA3 recurrent axon collateral system, perhaps contributing to the limbic and tonic-clonic components of the observed epileptic phenotype. Axonal action potential conduction was altered as well in the sciatic nerve-a deficit potentially related to the pathophysiology of episodic ataxia/myokymia, a disease associated with missense mutations of the human K(v)1.1 gene.",
author = "Smart, {Sharon L.} and Valeri Lopantsev and Zhang, {C. L.} and Robbins, {Carol A.} and Hao Wang and Chiu, {S. Y.} and Schwartzkroin, {Philip A} and Albee Messing and Tempel, {Bruce L.}",
year = "1998",
month = "4",
doi = "10.1016/S0896-6273(00)81018-1",
language = "English (US)",
volume = "20",
pages = "809--819",
journal = "Neuron",
issn = "0896-6273",
publisher = "Cell Press",
number = "4",

}

TY - JOUR

T1 - Deletion of the K(v)1.1 Potassium channel causes epilepsy in mice

AU - Smart, Sharon L.

AU - Lopantsev, Valeri

AU - Zhang, C. L.

AU - Robbins, Carol A.

AU - Wang, Hao

AU - Chiu, S. Y.

AU - Schwartzkroin, Philip A

AU - Messing, Albee

AU - Tempel, Bruce L.

PY - 1998/4

Y1 - 1998/4

N2 - Mice lacking the voltage-gated potassium channel α subunit, K(v)1.1, display frequent spontaneous seizures throughout adult life. In hippocampal slices from homozygous K(v)1.1 null animals, intrinsic passive properties of CA3 pyramidal cells are normal. However, antidromic action potentials are recruited at lower thresholds in K(v)1.1 null slices. Furthermore, in a subset of slices, mossy fiber stimulation triggers synaptically mediated long-latency epileptiform burst discharges. These data indicate that loss of K(v)1.1 from its normal localization in axons and terminals of the CA3 region results in increased excitability in the CA3 recurrent axon collateral system, perhaps contributing to the limbic and tonic-clonic components of the observed epileptic phenotype. Axonal action potential conduction was altered as well in the sciatic nerve-a deficit potentially related to the pathophysiology of episodic ataxia/myokymia, a disease associated with missense mutations of the human K(v)1.1 gene.

AB - Mice lacking the voltage-gated potassium channel α subunit, K(v)1.1, display frequent spontaneous seizures throughout adult life. In hippocampal slices from homozygous K(v)1.1 null animals, intrinsic passive properties of CA3 pyramidal cells are normal. However, antidromic action potentials are recruited at lower thresholds in K(v)1.1 null slices. Furthermore, in a subset of slices, mossy fiber stimulation triggers synaptically mediated long-latency epileptiform burst discharges. These data indicate that loss of K(v)1.1 from its normal localization in axons and terminals of the CA3 region results in increased excitability in the CA3 recurrent axon collateral system, perhaps contributing to the limbic and tonic-clonic components of the observed epileptic phenotype. Axonal action potential conduction was altered as well in the sciatic nerve-a deficit potentially related to the pathophysiology of episodic ataxia/myokymia, a disease associated with missense mutations of the human K(v)1.1 gene.

UR - http://www.scopus.com/inward/record.url?scp=0032055649&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0032055649&partnerID=8YFLogxK

U2 - 10.1016/S0896-6273(00)81018-1

DO - 10.1016/S0896-6273(00)81018-1

M3 - Article

C2 - 9581771

AN - SCOPUS:0032055649

VL - 20

SP - 809

EP - 819

JO - Neuron

JF - Neuron

SN - 0896-6273

IS - 4

ER -