Prions and humans (A brief review of human prion diseases)

  • Srečko Koren University of Ljubljana, Medical Faculty, Institute of Microbiology and Immunology
Keywords: prions, prion diseases, prinew variant Creutzfeldt-Jakob disease

Abstract

Prion diseases, or transmissible spongiform encephalopathies are a group of neurodegenerative diseases that affect humans and animals. Prion diseases are manifest as infectious, genetic or sporadic disorders. They can be transmitted among different hosts by the infectious proteinaceous particles, called prions. Prions (PrP) are derived from a normal cellular isofrom PrPc after posttranslational conformational modification to PrPSc. New variant Creutzfeldt-Jakob disease (vCJD) is a novel human prion disorder with characteristic clinical and neuropathological features, which results from exposure to the prions causing bovine spongiform encephalopathy in cattle. Further studies are required to clarify risk factors and the possibility that the vCJD might spread from person to person by blood transfusion or by surgical procedures.

Presented at the International Conference: “Tell me what you eat, and I will tell you who you are – when BSE in Slovenia?”, 4-5 October 2000, Maribor, Slovenia

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Author Biography

Srečko Koren, University of Ljubljana, Medical Faculty, Institute of Microbiology and Immunology

Ljubljana, Slovenia. E-mail: srecko.koren@mf.uni-lj.si

References

Andrews NJ, Farrington CP, Cousens SN, Smith PG, Ward H, Knight RS, Ironside JW, Will RG Incidence of variant Creutzfeldt-Jakob disease in the UK. Lancet 2000; 356: 481-2.

Collinge J, Sidle KCL, Meads J, Ironside J, Hill AF. Molecular analysis of prion strain variation and the aetiology of new variant CJD. Nature 1996; 383: 685-90.

Harris DA. Cellular biology of prion diseases. Clin. Microbiol. Rev. 1999; 12: 429-44.

Hill AF, Zeidler M, Ironside J, Collinge J. Diagnosis of new variant Creutzfeldt-Jakob disease by tonsil biopsy. Lancet 1997; 349: 99-100.

Kitamoto T, Tateishi J. Human prion disease and human prion protein disease. In: Prusiner SB (ed.), Prions, Prions, Prions. Current Topics in Microbiology and Immunology 207, Springer-Verlag Berlin Heidelberg, 1996; 27-34.

Lasmezas CI, Deslys JP, Demainy R, Adjou KT, Lamoury F, Dormont D. BSE transmission to macaques. Nature 1996; 381: 743-4.

Prusiner SB. Scrapie prions. Annu. Rev. Microbiol. 1989; 43: 345-74.

Prusiner SB, Scott MR, De Armond SJ, Cohen FE. Prion protein biology. Cell 1998; 93: 337-48.

Prusiner SB. Human prion diseases. In: Zuckerman AJ, Bantavala JE, Pattison JR (eds.), Principles and Practice of Clinical Virology. 4th ed. John Wiley & Sons, Ltd., 2000; 711-47.

Soto C. Alzheimer’s and prion disease as disorders of protein conformation: implications for the design of novel therapeutic approaches. J. Mol. Med. 1999; 77: 412-8.

Will RG, Ironside JW, Zeidler M, Cousens SN, Estibeino K, Alperovitch A, Poser S, Pocchiari M, Hofman A, Smith PG. A new variant of Creutzfeldt-Jakob disease in the UK. Lancet 1996; 347: 921-5.

Published
2020-08-25
How to Cite
Koren S. (2020). Prions and humans (A brief review of human prion diseases). Agricultura, 1(1), 8-10. https://doi.org/10.18690/agricultura.1.1.8-10.2002
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Articles