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Prions cause degenerative neurological diseases-scrapie in animals and Creutzfeldt-lakob disease in humans. The novel properties and structure of the prion distinguish it from viruses. Introduction Scrapie is rapidly becoming one of the best understood degenerative neuro logical diseases. Progress in purification of the scrapie agent, or prion, over the last decade has led to an explosion of new information about slow infections causing brain degeneration. Perhaps the most unexpected discovery has been the identification of the cellular isoform of the scrapie prion protein (PrP) (1). While the function of this protein in normal cells is unknown, the presence of related PrP genes in virtually all eukaryotes ranging from yeast to humans suggests that cellular prion proteins play an important role in the metabolism of healthy cells. Only two years ago, all knowledge about the scrapie agent was derived from bioassays req uiring from two to [our months. Now, PrP monoclonal antibodies (2) as well as cloned PrP genes and cDNAs (1) are being used to study the genetic origin of prions, their mode of replication, and the pathogenetic mechanisms of prion diseases. The significance of prion research for understanding nontransmissible degenerative disorders such as Alzheimer's disease (AD) is
Annual Review of Medicine – Annual Reviews
Published: Feb 1, 1987
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