Shen Lydia, Dettmer Ulf
College of Arts & Sciences, Cornell University, Ithaca, NY 14853, USA.
Ann Romney Center for Neurologic Diseases, Brigham and Women's Hospital and Harvard Medical School, Boston, MA 02115, USA.
Biomolecules. 2024 Dec 22;14(12):1649. doi: 10.3390/biom14121649.
The maintenance of healthy mitochondria is essential for neuronal survival and relies upon mitochondrial quality control pathways involved in mitochondrial biogenesis, mitochondrial dynamics, and mitochondrial autophagy (mitophagy). Mitochondrial dysfunction is critically implicated in Parkinson's disease (PD), a brain disorder characterized by the progressive loss of dopaminergic neurons in the substantia nigra. Consequently, impaired mitochondrial quality control may play a key role in PD pathology. This is affirmed by work indicating that genes such as PRKN and PINK1, which participate in multiple mitochondrial processes, harbor PD-associated mutations. Furthermore, mitochondrial complex-I-inhibiting toxins like MPTP and rotenone are known to cause Parkinson-like symptoms. At the heart of PD is alpha-synuclein (αS), a small synaptic protein that misfolds and aggregates to form the disease's hallmark Lewy bodies. The specific mechanisms through which aggregated αS exerts its neurotoxicity are still unknown; however, given the vital role of both αS and mitochondria to PD, an understanding of how αS influences mitochondrial maintenance may be essential to elucidating PD pathogenesis and discovering future therapeutic targets. Here, the current knowledge of the relationship between αS and mitochondrial quality control pathways in PD is reviewed, highlighting recent findings regarding αS effects on mitochondrial biogenesis, dynamics, and autophagy.
维持健康的线粒体对于神经元存活至关重要,这依赖于参与线粒体生物发生、线粒体动力学和线粒体自噬(mitophagy)的线粒体质量控制途径。线粒体功能障碍与帕金森病(PD)密切相关,帕金森病是一种脑部疾病,其特征是黑质中多巴胺能神经元逐渐丧失。因此,线粒体质量控制受损可能在PD病理过程中起关键作用。有研究表明,参与多个线粒体过程的PRKN和PINK1等基因存在与PD相关的突变,这证实了上述观点。此外,已知MPTP和鱼藤酮等线粒体复合体I抑制毒素会导致帕金森样症状。PD的核心是α-突触核蛋白(αS),这是一种小的突触蛋白,会错误折叠并聚集形成该病标志性的路易小体。聚集的αS发挥神经毒性的具体机制尚不清楚;然而,鉴于αS和线粒体在PD中都起着至关重要的作用,了解αS如何影响线粒体维持对于阐明PD发病机制和发现未来治疗靶点可能至关重要。在此,本文综述了目前关于PD中αS与线粒体质量控制途径之间关系的知识,重点介绍了关于αS对线粒体生物发生、动力学和自噬影响的最新发现。