Isakson Center for Neurological Disease Research, Department of Physiology and Pharmacology, University of Georgia, Athens, GA, USA.
Mol Neurodegener. 2024 Nov 23;19(1):87. doi: 10.1186/s13024-024-00774-0.
Nuclear pore complexes (NPCs) are embedded in the nuclear envelope and facilitate the exchange of macromolecules between the nucleus and cytoplasm in eukaryotic cells. The dysfunction of the NPC and nuclear transport plays a significant role in aging and the pathogenesis of various neurodegenerative diseases. Common features among these neurodegenerative diseases, including Parkinson's disease (PD), encompass mitochondrial dysfunction, oxidative stress and the accumulation of insoluble protein aggregates in specific brain regions. The susceptibility of dopaminergic neurons to mitochondrial stress underscores the pivotal role of mitochondria in PD progression. Disruptions in mitochondrial-nuclear communication are exacerbated by aging and α-synuclein-induced oxidative stress in PD. The precise mechanisms underlying mitochondrial impairment-induced neurodegeneration in PD are still unclear. Evidence suggests that perturbations in dopaminergic neuronal nuclei are linked to PD-related neurodegeneration. These perturbations involve structural damage to the nuclear envelope and mislocalization of pivotal transcription factors, potentially driven by oxidative stress or α-synuclein pathology. The presence of protein aggregates, pathogenic mutations, and ongoing oxidative stress can exacerbate the dysfunction of NPCs, yet this mechanism remains understudied in the context of oxidative stress-induced PD. This review summarizes the link between mitochondrial dysfunction and dopaminergic neurodegeneration and outlines the current evidence for nuclear envelope and nuclear transport abnormalities in PD, particularly in oxidative stress. We highlight the potential role of nuclear pore and nucleocytoplasmic transport dysfunction in PD and stress the importance of systematically investigating NPC components in PD.
核孔复合体(NPC)嵌入核膜中,促进真核细胞核质间大分子的交换。NPC 功能障碍和核转运在衰老和各种神经退行性疾病的发病机制中起着重要作用。这些神经退行性疾病的共同特征包括帕金森病(PD),包括线粒体功能障碍、氧化应激和特定脑区不溶性蛋白聚集体的积累。多巴胺能神经元对线粒体应激的易感性强调了线粒体在 PD 进展中的关键作用。线粒体-核通讯的中断会因衰老和 PD 中α-突触核蛋白诱导的氧化应激而加剧。PD 中线粒体损伤诱导的神经退行性变的确切机制仍不清楚。有证据表明,多巴胺能神经元核的扰动与 PD 相关的神经退行性变有关。这些扰动涉及核膜的结构损伤和关键转录因子的定位错误,可能是由氧化应激或α-突触核蛋白病理学驱动的。蛋白聚集体、致病性突变和持续的氧化应激会加剧 NPC 的功能障碍,但这种机制在氧化应激诱导的 PD 中仍研究不足。本综述总结了线粒体功能障碍与多巴胺能神经退行性变之间的联系,并概述了 PD 中核膜和核转运异常的现有证据,特别是在氧化应激方面。我们强调了核孔和核质转运功能障碍在 PD 中的潜在作用,并强调系统研究 PD 中 NPC 成分的重要性。