Division of Biomedical Sciences, UCR School of Medicine, Riverside, California, United States.
Center for Glial-Neuronal Interaction, UCR School of Medicine, Riverside, California, United States.
ASN Neuro. 2020 Jan-Dec;12:1759091420979604. doi: 10.1177/1759091420979604.
While seizure disorders are more prevalent among multiple sclerosis (MS) patients than the population overall and prognosticate earlier death & disability, their etiology remains unclear. Translational data indicate perturbed expression of astrocytic molecules contributing to homeostatic neuronal excitability, including water channels (AQP4) and synaptic glutamate transporters (EAAT2), in a mouse model of MS with seizures (MS+S). However, astrocytes in MS+S have not been examined. To assess the translational relevance of astrocyte dysfunction observed in a mouse model of MS+S, demyelinated lesion burden, astrogliosis, and astrocytic biomarkers (AQP4/EAAT2/ connexin-CX43) were evaluated by immunohistochemistry in postmortem hippocampi from MS & MS+S donors. Lesion burden was comparable in MS & MS+S cohorts, but astrogliosis was elevated in MS+S CA1 with a concomitant decrease in EAAT2 signal intensity. AQP4 signal declined in MS+S CA1 & CA3 with a loss of perivascular AQP4 in CA1. CX43 expression was increased in CA3. Together, these data suggest that hippocampal astrocytes from MS+S patients display regional differences in expression of molecules associated with glutamate buffering and water homeostasis that could exacerbate neuronal hyperexcitability. Importantly, mislocalization of CA1 perivascular AQP4 seen in MS+S is analogous to epileptic hippocampi without a history of MS, suggesting convergent pathophysiology. Furthermore, as neuropathology was concentrated in MS+S CA1, future study is warranted to determine the pathophysiology driving regional differences in glial function in the context of seizures during demyelinating disease.
虽然癫痫发作障碍在多发性硬化症 (MS) 患者中的发病率高于一般人群,并预示着更早的死亡和残疾,但它们的病因仍不清楚。转化数据表明,在具有癫痫发作的 MS 小鼠模型 (MS+S) 中,星形胶质细胞分子的表达失调,包括水通道 (AQP4) 和突触谷氨酸转运体 (EAAT2),有助于神经元兴奋性的稳态。然而,MS+S 中的星形胶质细胞尚未被检测到。为了评估在 MS+S 小鼠模型中观察到的星形胶质细胞功能障碍的转化相关性,通过免疫组织化学评估了脱髓鞘病变负担、星形胶质细胞增生和星形胶质细胞生物标志物 (AQP4/EAAT2/连接蛋白-CX43) 在 MS 和 MS+S 供体的死后海马中。病变负担在 MS 和 MS+S 队列中是可比的,但 MS+S CA1 中的星形胶质细胞增生增加,同时 EAAT2 信号强度降低。AQP4 信号在 MS+S CA1 和 CA3 中下降,CA1 中的血管周 AQP4 丢失。CX43 表达在 CA3 中增加。总之,这些数据表明,来自 MS+S 患者的海马星形胶质细胞在与谷氨酸缓冲和水稳态相关的分子表达上显示出区域差异,这可能加剧神经元过度兴奋。重要的是,在 MS+S 中观察到的 CA1 血管周 AQP4 的定位错误类似于没有 MS 病史的癫痫海马,表明存在趋同的病理生理学。此外,由于神经病理学主要集中在 MS+S CA1 中,因此需要进一步研究以确定在脱髓鞘疾病期间癫痫发作时胶质细胞功能区域差异的病理生理学驱动因素。