Propagation and spread of pathogenic protein assemblies in neurodegenerative diseases.
Propagation and spread of pathogenic protein assemblies in neurodegenerative diseases.
复制标题
DOI：
10.1038/s41593-018-0238-6
复制
发表时间：
2018-10
影响因子：
25
通讯作者：
Walker LC
中科院分区：
文献类型：
--
作者：
Jucker M;Walker LC
许多神经退行性疾病，如阿尔茨海默病、帕金森氏病和肌萎缩侧索硬化症，其特征是神经系统中出现异常的蛋白质集合。在实验系统中的研究表明，这些组件来自特定错误折叠的蛋白质的Prion样种子聚集，这些蛋白质增殖并聚集形成每种疾病的典型细胞内和/或细胞外病变。蛋白质种子产生的寄主提供了支持或限制种子萌发、增殖、自组装和传播的生化和生理环境。多种机制影响种子的时空传播和所致病变的性质，其中之一是细胞摄取、释放和沿神经路径和网络运输种子。受影响网络中的细胞和区域的特征决定了它们的脆弱性，从而影响了疾病的神经病理和临床属性。因此，致病蛋白组件在神经系统内的传播由蛋白致病因子和宿主环境的相互作用决定。
Many neurodegenerative diseases such as Alzheimer’s disease, Parkinson’s disease, and amyotrophic lateral sclerosis are characterized by the progressive appearance of abnormal proteinaceous assemblies in the nervous system. Studies in experimental systems indicate that the assemblies originate from the prion-like seeded aggregation of specific misfolded proteins that proliferate and amass to form the intracellular and/or extracellular lesions that are typical of each disorder. The host in which the proteopathic seeds arise provides the biochemical and physiological environment that either supports or restricts their emergence, proliferation, self-assembly and spread. Multiple mechanisms influence the spatiotemporal spread of seeds and the nature of the resulting lesions, one of which is the cellular uptake, release, and transport of seeds along neural pathways and networks. The characteristics of cells and regions in the affected network govern their vulnerability and thereby influence the neuropathological and clinical attributes of the disease. The propagation of pathogenic protein assemblies within the nervous system thus is determined by the interaction of the proteopathic agent and the host milieu.
登录
查看更多内容
影响因子：
4.3
作者：
DeVos SL;Corjuc BT;Oakley DH;Nobuhara CK;Bannon RN;Chase A;Commins C;Gonzalez JA;Dooley PM;Frosch MP;Hyman BT
通讯作者：
Hyman BT
影响因子：
4.6
作者：
Di Fede G;Catania M;Maderna E;Ghidoni R;Benussi L;Tonoli E;Giaccone G;Moda F;Paterlini A;Campagnani I;Sorrentino S;Colombo L;Kubis A;Bistaffa E;Ghetti B;Tagliavini F
通讯作者：
Tagliavini F
影响因子：
6
作者：
Bolmont, Tristan;Clavaguera, Florence;Jucker, Mathias
通讯作者：
Jucker, Mathias
DOI：
10.1073/pnas.1206171109
发表时间：
2012-05-29
影响因子：
11.1
作者：
Busche, Marc Aurel;Chen, Xiaowei;Konnerth, Arthur
通讯作者：
Konnerth, Arthur
影响因子：
11.8
作者：
Brown P;Brandel JP;Sato T;Nakamura Y;MacKenzie J;Will RG;Ladogana A;Pocchiari M;Leschek EW;Schonberger LB
通讯作者：
Schonberger LB