Chen Chunling, Holth Jerrah K, Bunton-Stasyshyn Rosie, Anumonwo Charles K, Meisler Miriam H, Noebels Jeffrey L, Isom Lori L
Department of Pharmacology University of Michigan Medical School Ann Arbor Michigan 48109.
Department of Neurology Baylor College of Medicine Houston Texas 77030.
Ann Clin Transl Neurol. 2018 Jul 5;5(8):982-987. doi: 10.1002/acn3.599. eCollection 2018 Aug.
Deletion of , encoding the microtubule-binding protein Tau, prevents disease in multiple genetic models of hyperexcitability. To investigate whether the effect of Tau depletion is generalizable across multiple sodium channel gene-linked models of epilepsy, we examined the mouse model of Dravet syndrome, and the model of Early Infantile Epileptic Encephalopathy. Both models display severe seizures and early mortality. We found no prolongation of survival between , or mice or between , , or mice. Thus, the effect of deletion on mortality in epileptic encephalopathy models is gene specific and provides further mechanistic insight.
编码微管结合蛋白Tau的基因缺失可预防多种遗传性过度兴奋模型中的疾病。为了研究Tau缺失的影响是否在多种与钠通道基因相关的癫痫模型中具有普遍性，我们检测了Dravet综合征的小鼠模型和早期婴儿癫痫性脑病模型。这两种模型均表现出严重癫痫发作和早期死亡。我们发现，在野生型、Tau基因敲除或Tau基因敲入小鼠之间，以及在野生型、Scn1a基因敲除、Scn1a基因敲入小鼠之间，生存期均未延长。因此，基因缺失对癫痫性脑病模型死亡率的影响具有基因特异性，并提供了进一步的机制性见解。
