Benetti Federico, Geschwind Michael D, Legname Giuseppe
F1000 Biol Rep. 2010 Jun 23;2:46. doi: 10.3410/B2-46.
Prions are responsible for a heterogeneous group of fatal neurodegenerative diseases. They occur in three forms - sporadic, genetic, or acquired - and involve non-covalent post-translational modifications of the cellular prion protein (PrP(C)). Prions (PrP(Sc)) are characterized by their infectious properties and intrinsic ability to act as a template, converting the normal, physiological PrP(C) into the pathological form, PrP(Sc). The 'protein-only' hypothesis, postulated by Stanley B Prusiner, implies that the generation of de novo prions is possible. Exciting recent work, in vivo and in vitro, has further strengthened this postulate.
朊病毒是导致一组异质性致命神经退行性疾病的病原体。它们以三种形式出现——散发性、遗传性或获得性——并涉及细胞朊蛋白(PrP(C))的非共价翻译后修饰。朊病毒(PrP(Sc))的特征在于其传染性以及作为模板将正常的生理性PrP(C)转化为病理性形式PrP(Sc)的内在能力。由斯坦利·B·普鲁西纳提出的“仅蛋白质”假说是指有可能从头产生朊病毒。近期令人振奋的体内和体外研究工作进一步强化了这一假说。