Molecular biology of prions.
Molecular biology of prions.
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DOI：
10.55782/ane-2002-1434
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发表时间：
2002-09
影响因子：
1.4
通讯作者：
C. Weissmann;M. Enari;P. Klöhn;D. Rossi;E. Flechsig
中科院分区：
文献类型：
--
作者：
C. Weissmann;M. Enari;P. Klöhn;D. Rossi;E. Flechsig
“仅蛋白”假说认为，引起传染性海绵状脑病的传染因子是PrP的构象异构体，PrP是一种主要在大脑中表达的宿主蛋白，得到了许多证据的有力支持。到目前为止，朊病毒疾病在构象疾病中是独一无二的，因为它们不仅可以通过实验传播，而且可以通过自然途径传播，主要是通过摄入。朊病毒进入大脑的途径已大致阐明。朊病毒的一个显著特征是它们对常规消毒程序的非凡抵抗力，以及它们与金属和塑料表面结合而不失去传染性的能力。这种特性最初是在临床环境中观察到的，现在正在动物和细胞培养的实验环境中进行研究。
The "protein only" hypothesis holds that the infectious agent causing transmissible spongiform encephalopathies is a conformational isomer of PrP, a host protein predominantly expressed in brain and is strongly supported by many lines of evidence. Prion diseases are so far unique among conformational diseases in that they are transmissible, not only experimentally but also by natural routes, mainly by ingestion. The pathway of prions to the brain has been elucidated in outline. A striking feature of prions is their extraordinary resistance to conventional sterilisation procedures, and their capacity to bind to surfaces of metal and plastic without losing infectivity. This property, first observed in a clinical setting, is now being investigated in experimental settings, both in animals and in cell culture.