Eftekharzadeh Bahareh, Hyman Bradley T, Wegmann Susanne
Department of Neurology, Massachusetts General Hospital and Mass General Institute for Neurodegenerative Disease, Charlestown, MA 02129, USA.
Department of Neurology, Massachusetts General Hospital and Mass General Institute for Neurodegenerative Disease, Charlestown, MA 02129, USA.
Mech Ageing Dev. 2016 Jun;156:1-13. doi: 10.1016/j.mad.2016.03.001. Epub 2016 Mar 19.
The progression of many neurodegenerative diseases is assumed to be caused by misfolding of specific characteristic diseases related proteins, resulting in aggregation and fibril formation of these proteins. Protein misfolding associated age related diseases, although different in disease manifestations, share striking similarities. In all cases, one disease protein aggregates and loses its function or additionally shows a toxic gain of function. However, the clear link between these individual amyloid-like protein aggregates and cellular toxicity is often still uncertain. The similar features of protein misfolding and aggregation in this group of proteins, all involved in age related neurodegenerative diseases, results in high interest in characterization of their structural properties. We review here recent findings on structural properties of some age related disease proteins, in the context of their biological importance in disease.
许多神经退行性疾病的进展被认为是由特定特征性疾病相关蛋白的错误折叠引起的，导致这些蛋白聚集并形成纤维。与年龄相关的蛋白质错误折叠疾病，尽管在疾病表现上有所不同，但有显著的相似之处。在所有情况下，一种疾病蛋白聚集并失去其功能，或者额外表现出功能的毒性增加。然而，这些单个淀粉样蛋白聚集体与细胞毒性之间的明确联系往往仍不确定。这组蛋白质中蛋白质错误折叠和聚集的相似特征，都与年龄相关的神经退行性疾病有关，这使得人们对其结构特性的表征产生了浓厚兴趣。我们在此回顾一些与年龄相关疾病蛋白的结构特性的最新发现，以及它们在疾病中的生物学重要性。
