Prion diseases: a typical Kuhnian abnormality in a molecular paradigm.
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.
Silvestri
G
G
Department of Biochemistry and Biotechnology, Federico II University, Naples, Italy.
Baldassarre
F
F
D000818:Animals; D008954:Models, Biological; D010802:Phylogeny; D017096:Prion Diseases / Q000235:genetics*; D011328:Prions / Q000235:genetics
D011328:Prions