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Article

Increased susceptibility to cortical spreading depression in the mouse model of familial hemiplegic migraine type 2.

PLoS genetics - 1 Jun 2011

Leo Loredana, Gherardini Lisa, Barone Virginia, De Fusco Maurizio, Pietrobon Daniela, Pizzorusso Tommaso, Casari Giorgio

Abstract excerpt

Familial hemiplegic migraine type 2 (FHM2) is an autosomal dominant form of migraine with aura that is caused by mutations of the α2-subunit of the Na,K-ATPase, an isoform almost exclusively expressed in astrocytes in the adult brain. We generated the first FHM2 knock-in mouse model carrying the human W887R mutation in the Atp1a2 orthologous gene. Homozygous Atp1a2(R887/R887) mutants died just after birth, while...

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Identifiers

PMID: 21731499
DOI: 10.1371/journal.pgen.1002129
PMCID: PMC3121757

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