Widespread brain parenchymal HMGB1 and NF-κB neuroinflammatory responses upon cortical spreading depolarization in familial hemiplegic migraine type 1 mice
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چکیده
منابع مشابه
Enhanced subcortical spreading depression in familial hemiplegic migraine type 1 mutant mice.
Familial hemiplegic migraine type 1, a monogenic migraine variant with aura, is linked to gain-of-function mutations in the CACNA1A gene encoding Ca(V)2.1 channels. The S218L mutation causes severe channel dysfunction, and paroxysmal migraine attacks can be accompanied by seizures, coma, and hemiplegia; patients expressing the R192Q mutation exhibit hemiplegia only. Familial hemiplegic migraine...
متن کاملFamilial Hemiplegic Migraine and Spreading Depression
OBJECTIVE Familial hemiplegic migraine (FHM) is an autosomal dominantly inherited subtype of migraine with aura, characterized by transient neurological signs and symptoms. Typical hemiplegic migraine attacks start in the first or second decade of life. Some patients with FHM suffer from daily recurrent attacks since childhood. Results from extensive studies of cellular and animal models have i...
متن کاملfamilial hemiplegic migraine and spreading depression
how to cite this article: kazemi h, speckmann ej, gorji a. familial hemiplegic migraine and spreading depression. iran j child neurol. 2014 summer;8(3): 6-11. abstract objective familial hemiplegic migraine (fhm) is an autosomal dominantly inherited subtype of migraine with aura, characterized by transient neurological signs and symptoms. typical hemiplegic migraine attacks start in the first...
متن کاملIncreased Susceptibility to Cortical Spreading Depression in the Mouse Model of Familial Hemiplegic Migraine Type 2
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 ...
متن کاملRNA expression profiling in brains of familial hemiplegic migraine type 1 knock-in mice.
BACKGROUND Various CACNA1A missense mutations cause familial hemiplegic migraine type 1 (FHM1), a rare monogenic subtype of migraine with aura. FHM1 mutation R192Q is associated with pure hemiplegic migraine, whereas the S218L mutation causes hemiplegic migraine, cerebellar ataxia, seizures, and mild head trauma-induced brain edema. Transgenic knock-in (KI) migraine mouse models were generated ...
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ژورنال
عنوان ژورنال: Neurobiology of Disease
سال: 2021
ISSN: 0969-9961
DOI: 10.1016/j.nbd.2021.105424