Faculty of Medicine, Al-Azhar University, Cairo, Egypt.
Faculty of Medicine, Zagazig University, El-Sharkia, Egypt.
Environ Sci Pollut Res Int. 2020 Apr;27(11):11461-11483. doi: 10.1007/s11356-020-07914-1. Epub 2020 Feb 19.
Aggregation of particular proteins in the form of inclusion bodies or plaques followed by neuronal death is a hallmark of neurodegenerative proteopathies such as primary Parkinsonism, Alzheimer's disease, Lou Gehrig's disease, and Huntington's chorea. Complex polygenic and environmental factors implicated in these proteopathies. Accumulation of proteins in these disorders indicates a substantial disruption in protein homeostasis (proteostasis). Proteostasis or cellular proteome homeostasis is attained by the synchronization of a group of cellular mechanisms called the proteostasis network (PN), which is responsible for the stability of the proteome and achieves the equilibrium between synthesis, folding, and degradation of proteins. In this review, we will discuss the different types of PN and the impact of PN component dysfunction on the four major neurodegenerative diseases mentioned earlier. Graphical abstract.
特定蛋白质以包涵体或斑块的形式聚集，随后神经元死亡，这是神经退行性蛋白病的标志，如原发性帕金森病、阿尔茨海默病、葛雷克氏症和亨廷顿舞蹈症。这些蛋白病涉及复杂的多基因和环境因素。这些疾病中蛋白质的积累表明蛋白质平衡（蛋白质稳态）发生了实质性破坏。蛋白质稳态或细胞蛋白质组稳态是通过一组称为蛋白质稳态网络（PN）的细胞机制的同步实现的，该网络负责蛋白质组的稳定性，并在蛋白质的合成、折叠和降解之间实现平衡。在这篇综述中，我们将讨论不同类型的 PN 以及 PN 组件功能障碍对上述四种主要神经退行性疾病的影响。
