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Science, v.278 no.5336 = no.5336, 1997년, pp.245 - 251
Prusiner, Stanley B. (Department of Neurology (address for correspondence) and Department of Biochemistry and Biophysics, University of California, San Francisco, CA 94143, USA.)
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Bovine spongiform encephalopathy (BSE) and human Creutzfeldt-Jakob disease (CJD) are among the most notable central nervous system degenerative disorders caused by prions. CJD may present as a sporadic, genetic, or infectious illness. Prions are transmissible particles that are devoid of nucleic ac...
Prions are defined as proteinaceous infectious particles that lack nucleic acid. PrP C is the cellular prion protein; PrP Sc is the pathologic isoform. Amino-terminal truncation during limited proteolysis of PrP Sc produces PrP 27-30 (so named because this protease-resistant core of PrP Sc migrates at ∼27 to 30 kD).
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I thank F. E. Cohen and S. J. DeArmond for many stimulating discussions and their help in preparing this manuscript. Human specimens were kindly provided by J. Ironside R. Will and J. Bell. Supported by grants from NIH (NS14069 AG08967 P41-RR01081 AG02132 and NS22786) the International Human Frontiers of Science Program and the American Health Assistance Foundation as well as by gifts from the Sherman Fairchild Foundation the G. Harold and Leila Y. Mathers Foundation the Bernard Osher Foundation and Centeon Inc.
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