NMDA受体介导戊二酸尿症Ⅰ型纹状体神经元损伤

发布时间：2018-06-23 08:08

本文选题：戊二酸尿症Ⅰ型 + 纹状体　；参考：《临床儿科杂志》2017年10期

【摘要】：目的探讨NMDA受体在戊二酸尿症Ⅰ型纹状体神经元兴奋性损伤中的作用。方法利用构建的特异性沉默戊二酰辅酶A脱氢酶(GCDH)慢病毒载体感染原代培养纹状体神经元结合高浓度赖氨酸培养构建GA1细胞模型。Western-Blot检测NMDA受体蛋白表达水平变化。NMDA受体拮抗剂MK-801预处理神经元,再行慢病毒及高浓度赖氨酸干预,MTT检测神经元活性及Hoechst3342检测神经元凋亡情况。结果与对照组比较,实验组的NR2B蛋白表达升高,差异有统计学意义(P0.001)。实验组、对照组以及MK-801预处理组三组间神经元活性和正常核比例的差异均有统计学意义(P0.01);两两比较发现,实验组神经元活性和正常核比例较对照组明显下降,MK-801预处理后神经元活性和正常核比例较实验组明显升高,但仍低于对照组,差异均有统计学意义(P0.05)。结论 NR2B受体介导戊二酸尿症Ⅰ型体内代谢累积物致纹状体神经元损伤。
[Abstract]:Objective to investigate the role of NMDA receptor in the excitatory damage of type I striatal neurons in glutaric acid. Methods using the specific silenced two acyl coenzyme A dehydrogenase (GCDH) lentivirus vector to infect primary cultured striatum neurons with high concentration lysine culture and construct GA1 cell model.Western-Blot to detect the NMDA receptor protein table .NMDA receptor antagonist MK-801 pretreated neurons, followed by the intervention of lentivirus and high concentration lysine, MTT detection of neuron activity and Hoechst3342 detection of neuron apoptosis. Compared with the control group, the expression of NR2B protein in the experimental group was higher, the difference was statistically significant (P0.001). Experimental group, control group and MK-801 preposition There was significant difference in the activity of neuron and normal nucleus between the three groups (P0.01). 22 compared with the control group, the activity of neuron and normal nucleus in the experimental group were significantly lower than that of the control group. The ratio of neuron activity and normal nucleus after MK-801 pretreatment was significantly higher than that of the experimental group, but it was still lower than that in the control group, the difference was statistically significant (P 0.05) conclusion NR2B receptor mediates the damage of striatal neurons induced by glutaric acid type I metabolites in vivo.
【作者单位】：华中科技大学同济医学院附属同济医院儿科;
【分类号】：R725.9

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