Silvestri G, Baldassarre F
Department of Biochemistry and Biotechnology, Federico II University, Naples, Italy.
Med Hypotheses. 2000 Jan;54(1):69-71. doi: 10.1054/mehy.1998.0837.
As a new class of pathogens with unusual properties, prions have been implied in several spongiform encephalopathies mainly affecting farm animals (scrapie, mad-cow disease) and humans (kuru, Creutzfeldt-Jacob disease, fatal familial insomnia) (1). The term prions underlines the feature that the agents would merely consist of a protein (2), perhaps a pathological counterpart of a cellular constituent with poorly defined functions (3-5). In reviewing relevant literature, in order to test the validity of the 'protein-only hypothesis' for coherence and consistency with a molecular paradigm, we have met evident discrepancies, making prion diseases a typical abnormality in a Kuhnian sense. This paper summarizes and analyses the main features of the protein-only model.
作为一类具有特殊性质的新型病原体,朊病毒已被认为与几种主要影响家畜(羊瘙痒症、疯牛病)和人类(库鲁病、克雅氏病、致死性家族性失眠症)的海绵状脑病有关(1)。朊病毒这个术语强调了这样一个特征,即这些病原体可能仅由一种蛋白质组成(2),也许是一种功能定义不明确的细胞成分的病理对应物(3-5)。在回顾相关文献时,为了检验“仅蛋白质假说”与分子范式的连贯性和一致性的有效性,我们发现了明显的差异,这使得朊病毒疾病在库恩意义上成为一种典型的异常情况。本文总结并分析了仅蛋白质模型的主要特征。