First FHM3 mouse model shows spontaneous cortical spreading depolarizations

Abstract Here we show, for the first time, spontaneous cortical spreading depolarization (CSD) events – the electrophysiological correlate of the migraine aura – in animals by using the first generated familial hemiplegic migraine type 3 (FHM3) transgenic mouse model. The mutant mice express L263V‐m...

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Main Authors: Nico A. Jansen, Anisa Dehghani, Margot M. L. Linssen, Cor Breukel, Else A. Tolner, Arn M. J. M. van denMaagdenberg
Format: Article
Language:English
Published: Wiley 2020-01-01
Series:Annals of Clinical and Translational Neurology
Online Access:https://doi.org/10.1002/acn3.50971
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author Nico A. Jansen
Anisa Dehghani
Margot M. L. Linssen
Cor Breukel
Else A. Tolner
Arn M. J. M. van denMaagdenberg
author_facet Nico A. Jansen
Anisa Dehghani
Margot M. L. Linssen
Cor Breukel
Else A. Tolner
Arn M. J. M. van denMaagdenberg
author_sort Nico A. Jansen
collection DOAJ
description Abstract Here we show, for the first time, spontaneous cortical spreading depolarization (CSD) events – the electrophysiological correlate of the migraine aura – in animals by using the first generated familial hemiplegic migraine type 3 (FHM3) transgenic mouse model. The mutant mice express L263V‐mutated α1 subunits in voltage‐gated NaV1.1 sodium channels (Scn1aL263V). CSDs consistently propagated from visual to motor cortex, recapitulating what has been shown in patients with migraine with aura. This model may be valuable for the preclinical study of migraine with aura and other diseases in which spreading depolarization is a prominent feature.
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spelling doaj.art-b4f3e967b6bd4d2488da20753ba68eb92022-12-21T22:11:38ZengWileyAnnals of Clinical and Translational Neurology2328-95032020-01-017113213810.1002/acn3.50971First FHM3 mouse model shows spontaneous cortical spreading depolarizationsNico A. Jansen0Anisa Dehghani1Margot M. L. Linssen2Cor Breukel3Else A. Tolner4Arn M. J. M. van denMaagdenberg5Department of Human Genetics Leiden University Medical Center Leiden The NetherlandsDepartment of Human Genetics Leiden University Medical Center Leiden The NetherlandsDepartment of Human Genetics Leiden University Medical Center Leiden The NetherlandsDepartment of Human Genetics Leiden University Medical Center Leiden The NetherlandsDepartment of Human Genetics Leiden University Medical Center Leiden The NetherlandsDepartment of Human Genetics Leiden University Medical Center Leiden The NetherlandsAbstract Here we show, for the first time, spontaneous cortical spreading depolarization (CSD) events – the electrophysiological correlate of the migraine aura – in animals by using the first generated familial hemiplegic migraine type 3 (FHM3) transgenic mouse model. The mutant mice express L263V‐mutated α1 subunits in voltage‐gated NaV1.1 sodium channels (Scn1aL263V). CSDs consistently propagated from visual to motor cortex, recapitulating what has been shown in patients with migraine with aura. This model may be valuable for the preclinical study of migraine with aura and other diseases in which spreading depolarization is a prominent feature.https://doi.org/10.1002/acn3.50971
spellingShingle Nico A. Jansen
Anisa Dehghani
Margot M. L. Linssen
Cor Breukel
Else A. Tolner
Arn M. J. M. van denMaagdenberg
First FHM3 mouse model shows spontaneous cortical spreading depolarizations
Annals of Clinical and Translational Neurology
title First FHM3 mouse model shows spontaneous cortical spreading depolarizations
title_full First FHM3 mouse model shows spontaneous cortical spreading depolarizations
title_fullStr First FHM3 mouse model shows spontaneous cortical spreading depolarizations
title_full_unstemmed First FHM3 mouse model shows spontaneous cortical spreading depolarizations
title_short First FHM3 mouse model shows spontaneous cortical spreading depolarizations
title_sort first fhm3 mouse model shows spontaneous cortical spreading depolarizations
url https://doi.org/10.1002/acn3.50971
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AT corbreukel firstfhm3mousemodelshowsspontaneouscorticalspreadingdepolarizations
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