Farrand Ariana Q, Gregory Rebecca A, Bäckman Cristina M, Helke Kristi L, Boger Heather A
Department of Neuroscience and Center on Aging, Medical University of South Carolina, 173 Ashley Ave, BSB 403, MSC 510, Charleston, SC 29425, USA.
Department of Neuroscience and Center on Aging, Medical University of South Carolina, 173 Ashley Ave, BSB 403, MSC 510, Charleston, SC 29425, USA; Department of Comparative Medicine, Medical University of South Carolina, 114 Doughty St, STB 648, MSC 777, Charleston, SC 29425, USA.
Brain Res. 2016 Nov 15;1651:88-94. doi: 10.1016/j.brainres.2016.09.025. Epub 2016 Sep 20.
Mitochondrial dysfunction has been implicated in the degeneration of dopamine (DA) neurons in Parkinson's disease (PD). In addition, animal models of PD utilizing neurotoxins, such as 6-hydroxydopamine and 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine, have shown that these toxins disrupt mitochondrial respiration by targeting complex I of the electron transport chain, thereby impairing DA neurons in these models. A MitoPark mouse model was created to mimic the mitochondrial dysfunction observed in the DA system of PD patients. These mice display the same phenotypic characteristics as PD, including accelerated decline in motor function and DAergic systems with age. Previously, these mice have responded to L-Dopa treatment and develop L-Dopa induced dyskinesia (LID) as they age. A potential mechanism involved in the formation of LID is greater glutamate release into the dorsal striatum as a result of altered basal ganglia neurocircuitry due to reduced nigrostriatal DA neurotransmission. Therefore, the focus of this study was to assess various indicators of glutamate neurotransmission in the dorsal striatum of MitoPark mice at an age in which nigrostriatal DA has degenerated. At 28 weeks of age, MitoPark mice had, upon KCl stimulation, greater glutamate release in the dorsal striatum compared to control mice. In addition, uptake kinetics were slower in MitoPark mice. These findings were coupled with reduced expression of the glutamate re-uptake transporter, GLT-1, thus providing an environment suitable for glutamate excitotoxic events, leading to altered physiological function in these mice.
线粒体功能障碍与帕金森病(PD)中多巴胺(DA)神经元的退化有关。此外,利用神经毒素(如6-羟基多巴胺和1-甲基-4-苯基-1,2,3,6-四氢吡啶)建立的PD动物模型表明,这些毒素通过靶向电子传递链的复合体I破坏线粒体呼吸,从而损害这些模型中的DA神经元。创建了MitoPark小鼠模型以模拟在PD患者DA系统中观察到的线粒体功能障碍。这些小鼠表现出与PD相同的表型特征,包括随着年龄增长运动功能和DA能系统加速衰退。此前,这些小鼠对左旋多巴治疗有反应,并随着年龄增长出现左旋多巴诱导的运动障碍(LID)。LID形成所涉及的一个潜在机制是,由于黑质纹状体DA神经传递减少导致基底神经节神经回路改变,从而使更多谷氨酸释放到背侧纹状体。因此,本研究的重点是评估在黑质纹状体DA已经退化的年龄阶段,MitoPark小鼠背侧纹状体中谷氨酸神经传递的各种指标。在28周龄时,与对照小鼠相比,MitoPark小鼠在氯化钾刺激下背侧纹状体中的谷氨酸释放更多。此外,MitoPark小鼠的摄取动力学较慢。这些发现与谷氨酸再摄取转运体GLT-1的表达降低有关,从而提供了一个适合谷氨酸兴奋性毒性事件的环境,导致这些小鼠的生理功能改变。