Kretzschmar H
Institut für Neuropathologie, Universität, Göttingen, Deutschland.
Wien Med Wochenschr. 1998;148(4):67-73.
Prion diseases are rare neurodegenerative and transmissible diseases affecting humans and mammals. The infectious agent of these deadly disease has been termed prion since in many respects this agent behaves differently from viruses. The prion hypothesis which was put forth by Stanley Prusiner in 1982 holds that the infectious agent consists of a conformationally changed normal cellular protein (PrPC). PrPC is a copper-binding protein of yet unknown function. PrPSc (the conformationally changed protein) in addition to its association with infectivity seems to have neurotoxic properties which are mediated by microglia in the CNS. Future research will have to concentrate on the dynamics of the conformational change from PrPC to PrPSc and understanding the neurotoxic mechanisms in prion diseases.
朊病毒疾病是影响人类和哺乳动物的罕见神经退行性和传染性疾病。由于在许多方面这种病原体的行为与病毒不同，这些致命疾病的传染性病原体被称为朊病毒。1982年斯坦利·普鲁西纳提出的朊病毒假说认为，传染性病原体由一种构象改变的正常细胞蛋白（PrPC）组成。PrPC是一种功能未知的铜结合蛋白。PrPSc（构象改变的蛋白）除了与传染性有关外，似乎还具有神经毒性特性，这种特性由中枢神经系统中的小胶质细胞介导。未来的研究将不得不专注于从PrPC到PrPSc的构象变化动态以及了解朊病毒疾病中的神经毒性机制。
