摘要
可传播性海绵状脑病是一类由朊病毒侵袭中枢神经系统而引起的致死性神经退行性疾病。在朊病毒病的病理过程中,细胞正常朊蛋白PrPC(cellular PrP)转化为异常构象的PrPSc(scrapie PrP)是至关重要的。目前,PrPSc转化增殖及致病机理仍不清楚。蛋白质错误折叠循环扩增(protein misfolding cyclic amplification,PMCA)技术的出现和应用,为研究朊病毒病的发病机制和发展敏感性高、特异性强的检测方法提供了很好的技术平台。
Prions are the infectious agents responsible for transmissible spongiform encephalopathies （TSEs）, which are a group of fatal neurodegenerative diseases. Although it appears that the critical event in the pathogenesis of prion disease is misfolding of the cellular prion protein （ PrP^C ） into a pathogenic isoform （PrP^Sc）, the pathogenic mechanism and conversion of PrP^Sc remain unknown. Protein misfolding cyclic amplification（PMCA） technology offers a great promise to develop a highly sensitive and specific method for the detection of prion and to further understand the molecular basis of prion propagation.
出处
《医学分子生物学杂志》
CAS
CSCD
2008年第6期550-553,共4页
Journal of Medical Molecular Biology
基金
国家科技支撑项目(No.2006BAD06A13-2)
国家重点基础研究发展规划(973计划) (No.2007CB310505)
国家自然科学基金(No.30571672
30500018
30771914)~~
关键词
可传播性海绵状脑病
朊病毒
蛋白错误折叠循环扩增
transmissible spongiform encephalopathies
prions
protein misfolding cyclic amplification
