路易体病和α-突触核蛋白病转基因模型中 mGluR5 表达和信号转导的改变——对兴奋性毒性的影响。

Alterations in mGluR5 expression and signaling in Lewy body disease and in transgenic models of alpha-synucleinopathy--implications for excitotoxicity.

机构信息

Department of Neurosciences, University of California San Diego, La Jolla, California, United States of America.

出版信息

PLoS One. 2010 Nov 16;5(11):e14020. doi: 10.1371/journal.pone.0014020.

Abstract

Dementia with Lewy bodies (DLB) and Parkinson's Disease (PD) are neurodegenerative disorders of the aging population characterized by the abnormal accumulation of alpha-synuclein (alpha-syn). Previous studies have suggested that excitotoxicity may contribute to neurodegeneration in these disorders, however the underlying mechanisms and their relationship to alpha-syn remain unclear. For this study we proposed that accumulation of alpha-syn might result in alterations in metabotropic glutamate receptors (mGluR), particularly mGluR5 which has been linked to deficits in murine models of PD. In this context, levels of mGluR5 were analyzed in the brains of PD and DLB human cases and alpha-syn transgenic (tg) mice and compared to age-matched, unimpaired controls, we report a 40% increase in the levels of mGluR5 and beta-arrestin immunoreactivity in the frontal cortex, hippocampus and putamen in DLB cases and in the putamen in PD cases. In the hippocampus, mGluR5 was more abundant in the CA3 region and co-localized with alpha-syn aggregates. Similarly, in the hippocampus and basal ganglia of alpha-syn tg mice, levels of mGluR5 were increased and mGluR5 and alpha-syn were co-localized and co-immunoprecipitated, suggesting that alpha-syn interferes with mGluR5 trafficking. The increased levels of mGluR5 were accompanied by a concomitant increase in the activation of downstream signaling components including ERK, Elk-1 and CREB. Consistent with the increased accumulation of alpha-syn and alterations in mGluR5 in cognitive- and motor-associated brain regions, these mice displayed impaired performance in the water maze and pole test, these behavioral alterations were reversed with the mGluR5 antagonist, MPEP. Taken together the results from study suggest that mGluR5 may directly interact with alpha-syn resulting in its over activation and that this over activation may contribute to excitotoxic cell death in select neuronal regions. These results highlight the therapeutic importance of mGluR5 antagonists in alpha-synucleinopathies.

摘要

路易体痴呆症 (DLB) 和帕金森病 (PD) 是老年人的神经退行性疾病,其特征是异常积聚α-突触核蛋白 (α-syn)。先前的研究表明,兴奋性毒性可能导致这些疾病的神经退行性变,但其潜在机制及其与α-syn 的关系仍不清楚。在这项研究中,我们提出α-syn 的积累可能导致代谢型谷氨酸受体 (mGluR) 的改变,特别是与 PD 小鼠模型缺陷相关的 mGluR5。在这种情况下,分析了 PD 和 DLB 人类病例以及α-syn 转基因 (tg) 小鼠脑中的 mGluR5 水平,并与年龄匹配的未受损对照进行了比较,我们报告说,在 DLB 病例的额叶皮质、海马体和纹状体以及 PD 病例的纹状体中,mGluR5 和β-arrestin 免疫反应性增加了 40%。在海马体中,mGluR5 在 CA3 区更为丰富,并与α-syn 聚集体共定位。同样,在α-syn tg 小鼠的海马体和基底节中,mGluR5 水平升高,mGluR5 和α-syn 共定位并共免疫沉淀,表明α-syn 干扰了 mGluR5 的运输。mGluR5 水平的增加伴随着下游信号转导成分(包括 ERK、Elk-1 和 CREB)的激活增加。与认知和运动相关脑区α-syn 的积累增加和 mGluR5 的改变一致,这些小鼠在水迷宫和杆测试中的表现受损,这些行为改变可被 mGluR5 拮抗剂 MPEP 逆转。总的来说,研究结果表明,mGluR5 可能与α-syn 直接相互作用,导致其过度激活,并且这种过度激活可能导致选择性神经元区域的兴奋性细胞死亡。这些结果强调了 mGluR5 拮抗剂在α-突触核蛋白病中的治疗重要性。

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