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下调泛素特异性蛋白酶 15(USP15)在实验性颞叶内侧癫痫中没有治疗益处。

Downregulation of Ubiquitin-Specific Protease 15 (USP15) Does Not Provide Therapeutic Benefit in Experimental Mesial Temporal Lobe Epilepsy.

机构信息

Experimental Epilepsy Research, Department of Neurosurgery, Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Breisacher Strasse 64, 79106, Freiburg, Germany.

BrainLinks-BrainTools Center, University of Freiburg, Georges-Koehler-Allee 201, 79110, Freiburg, Germany.

出版信息

Mol Neurobiol. 2024 Apr;61(4):2367-2389. doi: 10.1007/s12035-023-03692-2. Epub 2023 Oct 24.

Abstract

Structural epilepsies display complex immune activation signatures. However, it is unclear which neuroinflammatory pathways drive pathobiology. Transcriptome studies of brain resections from mesial temporal lobe epilepsy (mTLE) patients revealed a dysregulation of transforming growth factor β, interferon α/β, and nuclear factor erythroid 2-related factor 2 pathways. Since these pathways are regulated by ubiquitin-specific proteases (USP), in particular USP15, we hypothesized that USP15 blockade may provide therapeutic relief in treatment-resistant epilepsies. For validation, transgenic mice which either constitutively or inducibly lack Usp15 gene expression underwent intrahippocampal kainate injections to induce mTLE. We show that the severity of status epilepticus is unaltered in mice constitutively lacking Usp15 compared to wild types. Cell death, reactive gliosis, and changes in the inflammatory transcriptome were pronounced at 4 days after kainate injection. However, these brain inflammation signatures did not differ between genotypes. Likewise, induced deletion of Usp15 in chronic epilepsy did not affect seizure generation, cell death, gliosis, or the transcriptome. Concordantly, siRNA-mediated knockdown of Usp15 in a microglial cell line did not impact inflammatory responses in the form of cytokine release. Our data show that a lack of USP15 is insufficient to modulate the expression of relevant neuroinflammatory pathways in an mTLE mouse model and do not support targeting USP15 as a therapeutic approach for pharmacoresistant epilepsy.

摘要

结构性癫痫表现出复杂的免疫激活特征。然而,尚不清楚哪些神经炎症途径驱动病理生物学。对内侧颞叶癫痫 (mTLE) 患者脑切除标本的转录组研究显示,转化生长因子 β、干扰素 α/β 和核因子红细胞 2 相关因子 2 途径失调。由于这些途径受泛素特异性蛋白酶 (USP) 调节,特别是 USP15,我们假设 USP15 阻断可能为耐药性癫痫提供治疗缓解。为了验证这一点,我们对条件性或诱导性缺乏 Usp15 基因表达的转基因小鼠进行海马内海人酸注射以诱导 mTLE。我们表明,与野生型相比,条件性缺乏 Usp15 的小鼠癫痫持续状态的严重程度没有改变。在海人酸注射后 4 天,细胞死亡、反应性神经胶质增生和炎症转录组的变化明显。然而,这些大脑炎症特征在基因型之间没有差异。同样,慢性癫痫中诱导性缺失 Usp15 也不会影响发作发生、细胞死亡、神经胶质增生或转录组。同样,在小胶质细胞系中用 siRNA 介导的 Usp15 敲低不会影响细胞因子释放等形式的炎症反应。我们的数据表明,缺乏 USP15 不足以调节 mTLE 小鼠模型中相关神经炎症途径的表达,并且不支持将 USP15 作为治疗耐药性癫痫的方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8070/10973041/945bf2054df3/12035_2023_3692_Fig1_HTML.jpg

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