本文選題：戊二酸尿癥Ⅰ型 + 紋狀體��； 參考：《臨床兒科雜志》2017年10期

【摘要】：目的探討NMDA受體在戊二酸尿癥Ⅰ型紋狀體神經(jīng)元興奮性損傷中的作用。方法利用構(gòu)建的特異性沉默戊二酰輔酶A脫氫酶(GCDH)慢病毒載體感染原代培養(yǎng)紋狀體神經(jīng)元結(jié)合高濃度賴氨酸培養(yǎng)構(gòu)建GA1細胞模型。Western-Blot檢測NMDA受體蛋白表達水平變化。NMDA受體拮抗劑MK-801預(yù)處理神經(jīng)元,再行慢病毒及高濃度賴氨酸干預(yù),MTT檢測神經(jīng)元活性及Hoechst3342檢測神經(jīng)元凋亡情況。結(jié)果與對照組比較,實驗組的NR2B蛋白表達升高,差異有統(tǒng)計學(xué)意義(P0.001)。實驗組、對照組以及MK-801預(yù)處理組三組間神經(jīng)元活性和正常核比例的差異均有統(tǒng)計學(xué)意義(P0.01);兩兩比較發(fā)現(xiàn),實驗組神經(jīng)元活性和正常核比例較對照組明顯下降,MK-801預(yù)處理后神經(jīng)元活性和正常核比例較實驗組明顯升高,但仍低于對照組,差異均有統(tǒng)計學(xué)意義(P0.05)。結(jié)論 NR2B受體介導(dǎo)戊二酸尿癥Ⅰ型體內(nèi)代謝累積物致紋狀體神經(jīng)元損傷。
[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.
【作者單位】： 華中科技大學(xué)同濟醫(yī)學(xué)院附屬同濟醫(yī)院兒科;
【分類號】：R725.9

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