Department of Neurology, Philipps University, Baldingerstrasse, 35043, Marburg, Germany.
J Mol Neurosci. 2011 Nov;45(3):684-9. doi: 10.1007/s12031-011-9606-3. Epub 2011 Jul 27.
Progressive supranuclear palsy (PSP) is a sporadic and progressive neurodegenerative disease, most often leading to a symmetric, akinetic-rigid syndrome with prominent postural instability, vertical supranuclear gaze palsy, and cognitive decline. It belongs to the family of tauopathies and involves both cortical and subcortical structures. There is evidence from laboratory as well as in vivo studies suggesting that mitochondrial energy metabolism is impaired in PSP. Furthermore, several findings suggest that a failure in mitochondrial energy production might act as an upstream event in the chain of pathological events leading to the aggregation of tau and neuronal cell death. Agents targeting mitochondrial dysfunction have already shown a positive effect in a phase II study; however, further studies to verify these results need to be conducted. This review will focus on the pathophysiological concept of mitochondrial dysfunction in PSP and its possible role as a therapeutic target.
进行性核上性麻痹（PSP）是一种散发性进行性神经退行性疾病，最常导致对称、无动性僵硬综合征，伴有明显的姿势不稳、垂直核上性眼球运动障碍和认知功能下降。它属于 tau 病家族，涉及皮质和皮质下结构。实验室和体内研究均有证据表明，PSP 中线粒体能量代谢受损。此外，一些研究结果表明，线粒体能量产生的失败可能是导致 tau 聚集和神经元细胞死亡的病理事件链中的上游事件。针对线粒体功能障碍的药物已在 II 期研究中显示出积极效果；然而，需要进一步的研究来验证这些结果。这篇综述将重点介绍 PSP 中线粒体功能障碍的病理生理学概念及其作为治疗靶点的可能作用。
