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Prion diseases and the frame-shifting hypothesis

Authors: Wills PR
Publication: New Zealand Veterinary Journal, Volume 39, Issue 2, pp 41-45, Jun 1991
Publisher: Taylor and Francis

Animal type: General
Subject Terms: Nervous system/neurology, Spongiform encephalopathies, Disease/defect
Article class: Review Article

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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