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Procedures for Identifying Infectious Prions After Passage Through the Digestive System of an Avian Species
Published on: November 6, 2013
[Comments on present-day spread and epidemiology of BSE and prion diseases]
1Deutsches Primatenzentrum Göttingen, Abt. Infektionspathologie. bodemer@dpz.gwdg.de
Abstract:
Prion diseases of animals and man are neurological diseases with amyloidal deposition of the respective proteins. As to prion disease, the cellular prion protein is in its abnormal isoform(s) an essential component of prion protein aggregates found in affected tissue. In contrast to all neurodegenerative diseases like Morbus Alzheimer or Huntington's disease, prion diseases are transmissible. Therefore, prion diseases were designated Transmissible Spongiform Encephalopathies (TSE). The diseases have been well known for decades. Scrapie was first described around 1750, a BSE case was reported in the 1850-ties most likely a misdiagnosis, and in 1920/1930 the human Creutzfeldt-Jakob disease (CJD) had been described. Transmission of CJD i. e. Kuru had been suspected in the early 1950 s and was erroneously classified as slow virus disease. The CJD transmission posed a problem to humans when transplants from CJD cases were used for treatment. Fortunately, these iatrogenic transmissions remained limited. But with the advent of BSE and appearance of variant CJD cases in the UK and some places in Europe scientists suspected that transmission from cattle to man could have happened. From animal models we know of successful transmission via several routes. Species barriers do not completely prevent transmission. Rather, transmission barriers might exist controlling individual susceptibility against prions. Modes of transmission, susceptibility to transmission, identification of receptor molecules as well as molecular mechanisms of the transmission process are being investigated with great intensity. Current knowledge leads us to assume that inapparent stages of prion infection wrongly suggest a (non-existent) species barrier. This inapparent infection precedes overt disease, and, hence, most research focuses on the development of highly sensitive assay systems for detection of minute amounts of pathological prion protein in suspected cases. Inapparence also should warn us to underestimate BSE or human vCJD cases; at present, approx. 145 cases occurred in Europe and one probable case in Hong Kong (June 2003). Whether BSE had spread to other parts of the world by animal nutrition components or meat can neither be excluded nor confirmed at this time. New data on transmission and consequences of BSE for the human population are summarised in this review.
Insights
Prion diseases, or Transmissible Spongiform Encephalopathies (TSEs), are transmissible neurological disorders. Research intensifies on transmission routes, susceptibility, and detection of pathological prion protein, especially concerning Bovine Spongiform Encephalopathy (BSE).
Area of Science:
- Neuroscience
- Infectious Diseases
- Biochemistry
Background:
- Prion diseases are transmissible neurological disorders characterized by protein aggregation.
- Unlike other neurodegenerative diseases, prion diseases like Creutzfeldt-Jakob disease (CJD) and Bovine Spongiform Encephalopathy (BSE) are transmissible.
- Historical cases of Scrapie, BSE, and human CJD highlight the long-standing nature of these conditions.
Purpose of the Study:
- To review current knowledge on prion disease transmission, susceptibility, and molecular mechanisms.
- To highlight the challenges posed by inapparent prion infections and the need for sensitive detection methods.
- To summarize new data on BSE transmission and its implications for human health.
Main Methods:
- Review of existing literature on prion disease transmission and pathogenesis.
- Analysis of animal models to understand transmission routes and species barriers.
- Discussion of advancements in diagnostic assay systems for prion detection.
Main Results:
- Prion diseases are transmissible, with species barriers potentially modulating susceptibility rather than preventing transmission.
- Inapparent stages of infection can precede overt disease, complicating diagnosis and risk assessment.
- Bovine Spongiform Encephalopathy (BSE) transmission to humans (variant CJD) is a significant concern, with ongoing investigations into its spread.
Conclusions:
- Understanding prion transmission, including routes and susceptibility factors, is crucial for public health.
- Development of highly sensitive assays is essential for early detection of pathological prion protein.
- Continued research is needed to fully assess the global impact of BSE and prevent further human cases.
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