Victoria Guiliana Soraya, Zurzolo Chiara
Unité Trafic Membranaire et Pathogenese, Institut Pasteur, 25-28 Rue du Docteur Roux, 75724 Paris CEDEX 15, France.
Unité Trafic Membranaire et Pathogenese, Institut Pasteur, 25-28 Rue du Docteur Roux, 75724 Paris CEDEX 15, France.
Virus Res. 2015 Sep 2;207:146-54. doi: 10.1016/j.virusres.2015.01.019. Epub 2015 Jan 30.
Several neurodegenerative diseases such as transmissible spongiform encephalopathies, Alzheimer's and Parkinson's diseases are caused by the conversion of cellular proteins to a pathogenic conformer. Despite differences in the primary structure and subcellular localization of these proteins, which include the prion protein, α-synuclein and amyloid precursor protein (APP), striking similarity has been observed in their ability to seed and convert naïve protein molecules as well as transfer between cells. This review aims to cover what is known about the intracellular trafficking of these proteins as well as their degradation mechanisms and highlight similarities in their movement through the endocytic pathway that could contribute to the pathogenic conversion and seeding of these proteins which underlies the basis of these diseases.
几种神经退行性疾病，如传染性海绵状脑病、阿尔茨海默病和帕金森病，是由细胞蛋白转化为致病构象异构体引起的。尽管这些蛋白质的一级结构和亚细胞定位存在差异，包括朊病毒蛋白、α-突触核蛋白和淀粉样前体蛋白（APP），但在它们引发和转化未成熟蛋白分子以及在细胞间转移的能力方面，已观察到显著的相似性。本综述旨在涵盖关于这些蛋白质细胞内运输及其降解机制的已知信息，并强调它们在内吞途径中移动的相似性，这些相似性可能有助于这些蛋白质的致病转化和引发，而这正是这些疾病的基础。
