Department of Biochemistry, Institute of Biomedical & Health Sciences, Hiroshima University, 1-2-3 Kasumi, Minami-ku, Hiroshima, 734-8553, Japan.
Neurochem Res. 2022 Sep;47(9):2478-2487. doi: 10.1007/s11064-022-03608-x. Epub 2022 Apr 29.
The nuclear envelope (NE) separates genomic DNA from the cytoplasm and provides the molecular platforms for nucleocytoplasmic transport, higher-order chromatin organization, and physical links between the nucleus and cytoskeleton. Recent studies have shown that the NE is often damaged by various stresses termed "NE stress", leading to critical cellular dysfunction. Accumulating evidence has revealed the crucial roles of NE stress in the pathology of a broad spectrum of diseases. In the central nervous system (CNS), NE dysfunction impairs neural development and is associated with several neurological disorders, such as Alzheimer's disease and autosomal dominant leukodystrophy. In this review, the structure and functions of the NE are summarized, and the concepts of NE stress and NE stress responses are introduced. Additionally, the significant roles of the NE in the development of CNS and the mechanistic connections between NE stress and neurological disorders are described.
核膜（NE）将基因组 DNA 与细胞质分隔开，并为核质转运、高级染色质组织以及核与细胞骨架之间的物理连接提供了分子平台。最近的研究表明，NE 经常受到各种应激的破坏，这些应激被称为“NE 应激”，导致严重的细胞功能障碍。越来越多的证据表明，NE 应激在广泛疾病的病理学中起着关键作用。在中枢神经系统（CNS）中，NE 功能障碍会损害神经发育，并与几种神经退行性疾病有关，如阿尔茨海默病和常染色体显性遗传性脑白质营养不良。在这篇综述中，总结了 NE 的结构和功能，并介绍了 NE 应激和 NE 应激反应的概念。此外，还描述了 NE 在 CNS 发育中的重要作用以及 NE 应激与神经退行性疾病之间的机制联系。
