Targeting prion-like protein spreading in neurodegenerative diseases  

作　　者：Zhaohui Zhang Shuke Nie Liam Chen 

机构地区：[1]Department of Neurology,Renmin Hospital,Wuhan University [2]Department of Pathology,Johns Hopkins University School of Medicine

出　　处：《Neural Regeneration Research》2018年第11期1875-1878,共4页中国神经再生研究（英文版）

摘　　要：The infectious template-mediated protein conversion is a unique mechanism for the onset of rare and fatal neurodegenerative disorders known as transmissible spongiform encephalopathies, or prion diseases, which affect humans and other animal species. However, emerging studies are now demonstrating prion-like mechanisms of self-propagation of protein misfolding in a number of common, non-infectious neurodegenerative diseases such as Alzheimer＇s disease and Parkinson＇s disease. It has been proposed that distinct and unrelated proteins（beta-amyloid, tau, α-synuclein, TAR DNA-binding protein 43 and huntingtin, etc.） associated with common neurodegenerative disorders can seed conversion and spread via cellto-cell transfer, sustaining the transmission of neurotoxic agents along a stereotypic route, sharing features at the heart of the intrinsic nature of prions. Here we review the most recent development on both the molecular mechanisms underlying the pathogenesis of prion-like neurodegenerative diseases as well as innovative methods and strategies for potential therapeutic applications.The infectious template-mediated protein conversion is a unique mechanism for the onset of rare and fatal neurodegenerative disorders known as transmissible spongiform encephalopathies, or prion diseases, which affect humans and other animal species. However, emerging studies are now demonstrating prion-like mechanisms of self-propagation of protein misfolding in a number of common, non-infectious neurodegenerative diseases such as Alzheimer＇s disease and Parkinson＇s disease. It has been proposed that distinct and unrelated proteins（beta-amyloid, tau, α-synuclein, TAR DNA-binding protein 43 and huntingtin, etc.） associated with common neurodegenerative disorders can seed conversion and spread via cellto-cell transfer, sustaining the transmission of neurotoxic agents along a stereotypic route, sharing features at the heart of the intrinsic nature of prions. Here we review the most recent development on both the molecular mechanisms underlying the pathogenesis of prion-like neurodegenerative diseases as well as innovative methods and strategies for potential therapeutic applications.

关 键 词：prion-like SYNUCLEIN tau TAR DNA-binding protein 43 BETA-AMYLOID Parkinson＇s disease frontotemporal dementia amyotrophic lateral sclerosis Alzheimer＇s disease NEURODEGENERATION 

分 类 号：R741[医药卫生—神经病学与精神病学]