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Review Article

Prion diseases and the frame-shifting hypothesis

Pages 41-45 | Accepted 22 Jul 1991, Published online: 22 Feb 2011
 

Abstract

A group of neurological diseases, which includes scrapie, bovine spongiform encephalopathy and Creutzfeldt-Jakob disease, is caused by prion agents which appear to be comprised solely of an abnormal variant of a cellular protein, PrP. Infectious agents which contain no genetic material pose a conundrum for molecular biologists. Theoretical analysis of the gene encoding PrP has suggested a possible mechanism of replication for this proteinaceous infectious agent. The putative mechanism involves frame-shifting during translation of the PrP gene. The normal and abnormal forms of PrP are predicted to have different amino acid sequences.

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