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Nature 402, 737-739 (16 December 1999) | doi:10.1038/45413
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Spongiform encephalopathies: Breech-birth prions
James Hope
Creutzfeldt–Jakob disease, Gerstmann–Straussler–Scheinker (GSS) syndrome, scrapie and bovine spongiform encephalopathy are part of a family of neurodegenerative diseases caused by changes in the metabolism of a plasma-membrane protein, PrPC or prion protein. These prion diseases can be acquired by infection, arise sporadically by an unknown mechanism, or result from inheritance of a defective PrP gene.
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