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Letters
October 13, 1999

Some Implications of the Prion Paradigm: Caveat Denaturor

Author Affiliations
 

Margaret A.WinkerMD, Deputy EditorIndividualAuthorPhil B.FontanarosaMD, Interim CoeditorIndividualAuthor

JAMA. 1999;282(14):1332-1333. doi:10-1001/pubs.JAMA-ISSN-0098-7484-282-14-jbk1013

To the Editor: The term prion connotes an iconoclastic paradigm for an infectious agent afflicting humans and other animals.13 It imputes pathogenicity to the 3-dimensional shape of the PrPsc (prion protein scrapie). The normal cellular protein, PrPc, by exposure to PrPsc, adopts the SC conformation, maintaining a cascade of SC production in vivo. Unlike other self-replicating infectious viruses and bacteria, no unique sequences of DNA or RNA are necessary for this alteration, as the amino acid sequence of PrPc and PrPscare identical. The SC isoform does exhibit protease resistance as 1 marker. How this conformer modulation occurs is obscure; it may be similar to the "chaperone" functions of some auxiliary proteins that facilitate folding, or to the "epitaxial" outgrowth from a single crystal face, which is exploited in high-performance metallurgy. The propagation of prions is formally similar to that of centrioles, which are aggregates of tubulin that can re-form spontaneously, although they are typically propagated from existing centrioles.4

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