Cluster of Excellence NeuroCure, University Medicine Charité Berlin, Germany ; Department of Experimental Neurology, University Medicine Charité Berlin, Germany.
Front Neural Circuits. 2013 Nov 26;7:188. doi: 10.3389/fncir.2013.00188. eCollection 2013.
The extracellular concentration of the two main neurotransmitters glutamate and GABA is low but not negligible which enables a number of tonic actions. The effects of ambient GABA vary in a region-, cell-type, and age-dependent manner and can serve as indicators of disease-related alterations. Here we explored the tonic inhibitory actions of GABA in Huntington's disease (HD). HD is a devastating neurodegenerative disorder caused by a mutation in the huntingtin gene. Whole cell patch clamp recordings from striatal output neurons (SONs) in slices from adult wild type mice and two mouse models of HD (Z_Q175_KI homozygotes or R6/2 heterozygotes) revealed an HD-related reduction of the GABA(A) receptor-mediated tonic chloride current (I(Tonic(GABA))) along with signs of reduced GABA(B) receptor-mediated presynaptic depression of synaptic GABA release. About half of I(Tonic(GABA)) depended on tetrodotoxin-sensitive synaptic GABA release, but the remaining current was still lower in HD. Both in WT and HD, I(Tonic(GABA)) was more prominent during the first 4 h after preparing the slices, when astrocytes but not neurons exhibited a transient depolarization. All further tests were performed within 1-4 h in vitro. Experiments with SNAP5114, a blocker of the astrocytic GABA transporter GAT-3, suggest that in WT but not HD GAT-3 operated in the releasing mode. Application of a transportable substrate for glutamate transporters (D-aspartate 0.1-1 mM) restored the non-synaptic GABA release in slices from HD mice. I(Tonic(GABA)) was also rescued by applying the hyperagonist gaboxadol (0.33 μM). The results lead to the hypothesis that lesion-induced astrocyte depolarization facilitates non-synaptic release of GABA through GAT-3. However, the capacity of depolarized astrocytes to provide GABA for tonic inhibition is strongly reduced in HD.
两种主要神经递质谷氨酸和 GABA 的细胞外浓度较低,但并非可以忽略不计,这使其能够发挥多种紧张作用。周围 GABA 的作用因区域、细胞类型和年龄而异,可作为与疾病相关变化的指标。在这里,我们探索了 GABA 在亨廷顿病(HD)中的紧张抑制作用。HD 是一种由亨廷顿基因突变引起的破坏性神经退行性疾病。来自成年野生型小鼠和两种 HD 小鼠模型(Z_Q175_KI 纯合子或 R6/2 杂合子)脑片的纹状体输出神经元(SONs)的全细胞膜片钳记录显示,GABA(A) 受体介导的紧张氯离子电流(I(Tonic(GABA)))与 GABA(B) 受体介导的突触 GABA 释放的 presynaptic 抑制减少有关,表明与 HD 相关的减少。大约一半的 I(Tonic(GABA)))取决于河豚毒素敏感的突触 GABA 释放,但在 HD 中剩余的电流仍然较低。在 WT 和 HD 中,I(Tonic(GABA)))在切片制备后的头 4 小时内更为明显,此时星形胶质细胞而非神经元表现出短暂去极化。所有进一步的测试均在体外 1-4 小时内进行。用 SNAP5114(星形胶质细胞 GABA 转运蛋白 GAT-3 的阻断剂)进行的实验表明,在 WT 中,但不在 HD 中,GAT-3 以释放模式起作用。应用谷氨酸转运体的可运输底物(D-天冬氨酸 0.1-1 mM)可恢复 HD 小鼠脑片中的非突触 GABA 释放。应用超激动剂 gaboxadol(0.33 μM)也可以挽救 I(Tonic(GABA)))。这些结果导致假设损伤诱导的星形胶质细胞去极化通过 GAT-3 促进非突触 GABA 释放。然而,去极化星形胶质细胞为紧张抑制提供 GABA 的能力在 HD 中大大降低。