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First FHM3 mouse model shows spontaneous cortical spreading depolarizations.
Annals of Clinical and Translational Neurology ( IF 4.4 ) Pub Date : 2019-12-27 , DOI: 10.1002/acn3.50971
Nico A Jansen 1 , Anisa Dehghani 1 , Margot M L Linssen 1 , Cor Breukel 1 , Else A Tolner 1, 2 , Arn M J M van den Maagdenberg 1, 2
Affiliation  

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.

中文翻译:

第一个FHM3小鼠模型显示自发性皮层扩张去极化。

在这里,我们首次使用第一个产生的家族性偏瘫3型偏头痛(FHM3)转基因小鼠模型,首次显示了动物自发性皮层扩展去极化(CSD)事件–偏头痛先兆的电生理相关性。突变小鼠表达L263V突变α在电压门控Na 1个亚基V 1.1钠通道(SCN1A L263V)。CSD从视觉皮层一直传播到运动皮层,概括了偏头痛先兆患者的表现。该模型对于偏头痛先兆和其他疾病的临床前研究可能有价值,在这些疾病中,传播去极化是一个突出特征。
更新日期:2019-12-27
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