Joshi Neha, Raveendran Atchaya, Nagotu Shirisha
Organelle Biology and Cellular Ageing Lab, Department of Biosciences and Bioengineering, Indian Institute of Technology Guwahati, Guwahati 781039, Assam, India.
Diseases. 2020 Jun 22;8(2):24. doi: 10.3390/diseases8020024.
Proper folding to attain a defined three-dimensional structure is a prerequisite for the functionality of a protein. Improper folding that eventually leads to formation of protein aggregates is a hallmark of several neurodegenerative disorders. Loss of protein homeostasis triggered by cellular stress conditions is a major contributing factor for the formation of these toxic aggregates. A conserved class of proteins called chaperones and co-chaperones is implicated in maintaining the cellular protein homeostasis. Expanding the body of evidence highlights the role of chaperones as central mediators in the formation, de-aggregation and degradation of the aggregates. Altered expression and function of chaperones is associated with many neurodegenerative diseases including Parkinson's disease. Several studies indicate that chaperones are at the center of the cause and effect cycle of this disease. An overview of the various chaperones that are associated with homeostasis of Parkinson's disease-related proteins and their role in pathogenicity will be discussed in this review.
正确折叠以获得确定的三维结构是蛋白质发挥功能的前提条件。最终导致蛋白质聚集体形成的错误折叠是几种神经退行性疾病的标志。细胞应激条件引发的蛋白质稳态丧失是这些有毒聚集体形成的主要促成因素。一类被称为伴侣蛋白和共伴侣蛋白的保守蛋白质与维持细胞蛋白质稳态有关。越来越多的证据表明，伴侣蛋白在聚集体的形成、解聚和降解中起着核心介导作用。伴侣蛋白表达和功能的改变与包括帕金森病在内的许多神经退行性疾病有关。几项研究表明，伴侣蛋白处于这种疾病因果循环的中心。本文将综述与帕金森病相关蛋白质稳态相关的各种伴侣蛋白及其在致病性中的作用。
