Open AccessFirst FHM3 mouse model shows spontaneous cortical spreading depolarizations
Author(s) -
Jansen Nico A.,
Dehghani Anisa,
Linssen Margot M. L.,
Breukel Cor,
Tolner Else A.,
Maagdenberg Arn M. J. M.
Publication year - 2020
Publication title -
annals of clinical and translational neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.824
H-Index - 42
ISSN - 2328-9503
DOI - 10.1002/acn3.50971
Subject(s) - familial hemiplegic migraine , depolarization , cortical spreading depression , migraine , neuroscience , aura , migraine with aura , medicine , sodium channel , electrophysiology , cortex (anatomy) , genetically modified mouse , cortical neurons , neocortex , visual cortex , transgene , biology , anesthesia , sodium , chemistry , gene , genetics , organic chemistry
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 α 1 subunits in voltage‐gated Na V 1.1 sodium channels ( Scn1a L263V ). 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.