FREE NEJM E-TOC    HOME   |   SUBSCRIBE   |   CURRENT ISSUE   |   PAST ISSUES   |   COLLECTIONS   |   Search Term  Advanced Search

Sign in | Get NEJM's E-Mail Table of Contents — Free | Subscribe

Previous Volume 312:73-78 January 10, 1985 Number 2 Next

	Add to Personal Archive Add to Citation Manager Notify a Friend E-mail When Cited PubMed Citation

Abstract

Creutzfeldt-Jakob disease is caused by a slow infectious pathogen, or prion. We found that purified fractions from the brains of two patients with Creutzfeldt-Jakob disease contained protease-resistant proteins ranging in apparent molecular weight from 10,000 to 50,000. These proteins reacted with antibodies raised against the scrapie prion protein PrP 27-30. Rod-shaped particles were found in the brain tissue of the patients that were similar to those isolated from rodents with either scrapie or experimental Creutzfeldt-Jakob disease. After being stained with Congo red dye, the protein polymers from patients with Creutzfeldt-Jakob disease exhibited green birefringence when examined under polarized light. Our findings suggest that the amyloid plaques found in the brains of patients with Creutzfeldt-Jakob disease may be composed of paracrystalline arrays of prions similar to those in prion diseases in laboratory animals.

This article has been cited by other articles:

• Safar, J. G., Wille, H., Geschwind, M. D., Deering, C., Latawiec, D., Serban, A., King, D. J., Legname, G., Weisgraber, K. H., Mahley, R. W., Miller, B. L., DeArmond, S. J., Prusiner, S. B. (2006). Human prions and plasma lipoproteins. Proc. Natl. Acad. Sci. USA 103: 11312-11317 [Abstract] [Full Text]  
• Peretz, D., Supattapone, S., Giles, K., Vergara, J., Freyman, Y., Lessard, P., Safar, J. G., Glidden, D. V., McCulloch, C., Nguyen, H.-O. B., Scott, M., DeArmond, S. J., Prusiner, S. B. (2006). Inactivation of Prions by Acidic Sodium Dodecyl Sulfate. J. Virol. 80: 322-331 [Abstract] [Full Text]  
• Kobayashi, A., Satoh, S., Ironside, J. W., Mohri, S., Kitamoto, T. (2005). Type 1 and type 2 human PrPSc have different aggregation sizes in methionine homozygotes with sporadic, iatrogenic and variant Creutzfeldt-Jakob disease. J. Gen. Virol. 86: 237-240 [Abstract] [Full Text]  
• Prusiner, S. B. (1998). Prions. Proc. Natl. Acad. Sci. USA 95: 13363-13383 [Abstract] [Full Text]  
• Shetty, A. K., Steele, R. W. (1997). Commentary Review: Prion Diseases. CLIN PEDIATR 36: 1-7  
• Kretzschmar, H. A., Ironside, J. W., DeArmond, S. J., Tateishi, J. (1996). Diagnostic Criteria for Sporadic Creutzfeldt-Jakob Disease. Arch Neurol 53: 913-920 [Abstract]  
• Prusiner, S. B. (1993). Genetic and Infectious Prion Diseases. Arch Neurol 50: 1129-1153 [Abstract]  
• Hecker, R, Taraboulos, A, Scott, M, Pan, K M, Yang, S L, Torchia, M, Jendroska, K, DeArmond, S J, Prusiner, S B (1992). Replication of distinct scrapie prion isolates is region specific in brains of transgenic mice and hamsters.. Genes Dev. 6: 1213-1228 [Abstract]  
• Prusiner, S. (1991). Molecular biology of prion diseases. Science 252: 1515-1522 [Abstract]  
• Fraser, C. L., Arieff, A. I. (1988). Nervous System Complications in Uremia. ANN INTERN MED 109: 143-153 [Abstract]