Intellectual and Developmental Disabilities Research Center, Semel Institute for Neuroscience and Human Behavior, Brain Research Institute, David Geffen School of Medicine, University of California Los Angeles, Los Angeles, CA, USA.
Research Services, VA Portland Health Care System, Oregon Health & Science University, Portland, OR, USA.
Neurobiol Dis. 2017 Dec;108:29-44. doi: 10.1016/j.nbd.2017.07.020. Epub 2017 Jul 27.
Huntington's disease (HD) is a fatal genetic disorder characterized by cell death of medium-sized spiny neurons (MSNs) in the striatum, traditionally attributed to excessive glutamate inputs and/or receptor sensitivity. While changes in corticostriatal projections have typically been studied in mouse models of HD, morphological and functional alterations in thalamostriatal projections have received less attention. In this study, an adeno-associated virus expressing channelrhodopsin-2 under the calcium/calmodulin-dependent protein kinase IIα promoter was injected into the sensorimotor cortex or the thalamic centromedian-parafascicular nuclear complex in the R6/2 mouse model of HD, to permit selective activation of corticostriatal or thalamostriatal projections, respectively. In symptomatic R6/2 mice, peak amplitudes and areas of corticostriatal glutamate AMPA and NMDA receptor-mediated responses were reduced. In contrast, although peak amplitudes of AMPA and NMDA receptor-mediated thalamostriatal responses also were reduced, the areas remained unchanged due to an increase in response decay times. Blockade of glutamate reuptake further increased response areas and slowed rise and decay times of NMDA responses. These effects appeared more pronounced at thalamostriatal synapses of R6/2 mice, suggesting increased activation of extrasynaptic NMDA receptors. In addition, the probability of glutamate release was higher at thalamostriatal than corticostriatal synapses, particularly in R6/2 mice. Morphological studies indicated that the density of all excitatory synaptic contacts onto MSNs was reduced, which matches the basic electrophysiological findings of reduced amplitudes. There was a consistent reduction in the area of spines but little change in presynaptic terminal size, indicating that the postsynaptic spine may be more significantly affected than presynaptic terminals. These results highlight the significant and differential contribution of the thalamostriatal projection to glutamate excitotoxicity in HD.
亨廷顿病 (HD) 是一种致命的遗传性疾病,其特征是纹状体中型棘突神经元 (MSNs) 的细胞死亡,传统上归因于过度的谷氨酸输入和/或受体敏感性。虽然皮质纹状体投射的变化通常在 HD 的小鼠模型中进行研究,但丘脑纹状体投射的形态和功能改变受到的关注较少。在这项研究中,一种表达钙/钙调蛋白依赖性蛋白激酶 IIα启动子下的通道视紫红质-2 的腺相关病毒被注射到 R6/2 型 HD 小鼠模型的感觉运动皮层或丘脑中央中缝旁核复合体中,分别允许皮质纹状体或丘脑纹状体投射的选择性激活。在有症状的 R6/2 小鼠中,皮质纹状体谷氨酸 AMPA 和 NMDA 受体介导的反应的峰值幅度和面积减少。相比之下,尽管 AMPA 和 NMDA 受体介导的丘脑纹状体反应的峰值幅度也降低,但由于反应衰减时间的增加,面积保持不变。谷氨酸再摄取的阻断进一步增加了反应面积,并减缓了 NMDA 反应的上升和衰减时间。这些影响在 R6/2 型小鼠的丘脑纹状体突触中似乎更为明显,表明突触外 NMDA 受体的激活增加。此外,与皮质纹状体突触相比,谷氨酸释放的可能性在丘脑纹状体突触中更高,特别是在 R6/2 型小鼠中。形态学研究表明,所有兴奋性突触接触到 MSNs 的密度降低,这与幅度降低的基本电生理发现相匹配。棘突的面积普遍减少,但突触前末梢的大小变化不大,表明突触后棘突比突触前末梢受到的影响更大。这些结果突出了丘脑纹状体投射对 HD 中谷氨酸兴奋性毒性的显著和差异贡献。