PrP's Double Causes Trouble

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Science  29 Oct 1999:
Vol. 286, Issue 5441, pp. 914-915
DOI: 10.1126/science.286.5441.914

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Various strains of knockout mice--in which the gene that encodes the prion protein (PrP) has been disrupted--have been developed to study how PrP is involved in the prion diseases (which include scrapie, mad cow disease and Creutzfeldt-Jakob disease in humans). However, whereas some strains of PrP knockout mice get sick, others do not. This perplexing observation may now have been solved according to Weissmann and Aguzzi in their Perspective. They discuss a recent paper in the Journal of Molecular Biology that reports the discovery of a new Prnp-like gene called Prnd that may be expressed in the brains of some strains of knockout mice but not in others accounting for the variety in the phenotypes observed.