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Propagation of Protein Aggregation in Neurodegenerative Diseases.
Annual Review of Biochemistry ( IF 16.6 ) Pub Date : 2019-06-20 , DOI: 10.1146/annurev-biochem-061516-045049
Jaime Vaquer-Alicea 1 , Marc I Diamond 1
Affiliation  

Most common neurodegenerative diseases feature deposition of protein amyloids and degeneration of brain networks. Amyloids are ordered protein assemblies that can act as templates for their own replication through monomer addition. Evidence suggests that this characteristic may underlie the progression of pathology in neurodegenerative diseases. Many different amyloid proteins, including Aβ, tau, and α-synuclein, exhibit properties similar to those of infectious prion protein in experimental systems: discrete and self-replicating amyloid structures, transcellular propagation of aggregation, and transmissible neuropathology. This review discusses the contribution of prion phenomena and transcellular propagation to the progression of pathology in common neurodegenerative diseases such as Alzheimer's and Parkinson's. It reviews fundamental events such as cell entry, amplification, and transcellular movement. It also discusses amyloid strains, which produce distinct patterns of neuropathology and spread through the nervous system. These concepts may impact the development of new diagnostic and therapeutic strategies.

中文翻译:

神经退行性疾病中蛋白质聚集的传播。

最常见的神经退行性疾病以蛋白质淀粉样蛋白沉积和脑网络变性为特征。淀粉样蛋白是有序的蛋白质组装体,可通过添加单体作为自身复制的模板。有证据表明,该特征可能是神经退行性疾病病理进展的基础。许多不同的淀粉样蛋白,包括Aβ,tau和α-突触核蛋白,在实验系统中表现出与感染性ion病毒蛋白相似的特性:离散的和自我复制的淀粉样蛋白结构,聚集的跨细胞传播和可传播的神经病理学。这篇综述讨论了ion病毒现象和跨细胞传播对常见神经退行性疾病(例如阿尔茨海默氏病和帕金森氏病)病理学进展的贡献。它回顾了基本事件,例如细胞进入,扩增和跨细胞运动。它还讨论了淀粉样蛋白菌株,它们会产生不同的神经病理学模式并在神经系统中传播。这些概念可能会影响新的诊断和治疗策略的发展。
更新日期:2020-04-21
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