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Molecular Aspects of Disease Pathogenesis in the Transmissible Spongiform Encephalopathies

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Public Health Microbiology

Part of the book series: Methods in Molecular Biology ((MIMB,volume 268))

Abstract

The transmissible spongiform encephalopathy (TSE) diseases are a group of rare, fatal, and transmissible neurodegenerative diseases that include kuru and Creutzfeldt-Jakob disease (CJD) in humans, scrapie in sheep, transmissible mink encephalopathy (TME; 82), and chronic wasting disease (CWD) in mule deer (141) and elk (142). Over the last 20 yr, they have gone from a fascinating but relatively obscure group of diseases to one that is a major agricultural and economic problem as well as a threat to human health. The shift in the relative impact of the TSE diseases began in the late 1970s when the United Kingdom altered the process by which animal carcasses were rendered to provide a protein supplement (i.e., meat and bone meal) to sheep, cattle, and other livestock. Several years later a new disease was recognized in the British cattle population. The pathological and immunohistochemical characteristics of the disease clearly placed it among the TSEs. The new disease was named bovine spongiform encephalopathy (BSE) by the scientific community (2,16,138) and “mad cow disease” by the less-than-scientific press. At its peak in the UK, several thousand cattle a year were diagnosed with BSE, and millions of cattle were slaughtered. Introduction of the specified offals ban as well as banning the practice of feeding ruminants to other ruminants has led to a drastic decrease in the number of yearly BSE cases in the UK (less than 500 in 2003), and the epidemic is clearly on the wane. However, BSE has now spread throughout the rest of Europe, as well as to Japan, Russia, Canada, and Israel and thus remains a worldwide problem.

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Notes

  1. 1.

    *

    The normal form of PrP has been referred to as either PrPC for PrP-cellular or PrP-sen, which designates its sensitivity to proteinase K. The abnormal form of PrP has been referred to as either PrPSc for PrP-scrapie or PrP-res, which designates its partial resistance to proteinase K. Since the terms PrP-sen and PrP-res reflect a general operational definition rather than a disease-specific phenotype, they will be used throughout this discussion.

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Authors and Affiliations

  1. Institute for Virology, Technical University of Munich, Munich, Germany

    Suzette A. Priola

  2. Laboratory of Persistent Viral Diseases, National Institute of Allergy and Infectious Diseases, Rocky Mountain Laboratories, Hamilton, MT

    Ina Priola

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  1. Suzette A. Priola
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  2. Ina Priola
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  1. Planta Piloto de Procesos Industriales Microbiológicos, (PROIMI)-CONICET, San Miguel de Tucumán, Argentina

    John F. T. Spencer  & Alicia L. Ragout de Spencer  & 

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Priola, S.A., Priola, I. (2004). Molecular Aspects of Disease Pathogenesis in the Transmissible Spongiform Encephalopathies. In: Spencer, J.F.T., Ragout de Spencer, A.L. (eds) Public Health Microbiology. Methods in Molecular Biology, vol 268. Humana Press. https://doi.org/10.1385/1-59259-766-1:517

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  • DOI: https://doi.org/10.1385/1-59259-766-1:517

  • Publisher Name: Humana Press

  • Print ISBN: 978-1-58829-117-2

  • Online ISBN: 978-1-59259-766-6

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