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Updated: Jun 1, 2026
11:41
Assessing Transmissible Spongiform Encephalopathy Species Barriers with an In Vitro Prion Protein Conversion Assay
Published on: March 10, 2015
Atypical prion diseases in humans and animals
Michael A Tranulis1, Sylvie L Benestad, Thierry Baron
1Norwegian School of Veterinary Science, Oslo, Norway. Michael.Tranulis@nvh.no
Topics in Current Chemistry
|May 21, 2011
Summary
Prion diseases like CJD and scrapie are poorly understood due to long incubation periods. New atypical forms in animals may be linked to human prion diseases, raising public health concerns.
Area of Science:
- Neuroscience
- Veterinary Medicine
- Epidemiology
Background:
- Prion diseases, including Creutzfeldt-Jakob disease (CJD) in humans and scrapie in sheep, are recognized but their epidemiology and pathogenesis remain incompletely understood.
- Challenges in studying prion diseases include long incubation periods and difficulties in monitoring and characterizing the causative agents.
- Protease-resistant prion protein conformers (PrPSc) serve as disease markers, aiding in classification alongside clinical, pathological, and genetic data.
Purpose of the Study:
- To explore the nature of prion diseases, their transmission, and potential links between animal and human forms.
- To investigate the implications of newly identified atypical prion diseases in animals for public health.
- To enhance understanding of prion disease pathogenesis and epidemiology.
Main Methods:
- Analysis of clinical, pathological, and genetic data related to prion diseases.
- Characterization of protease-resistant prion protein conformers (PrPSc) as disease markers.
- Epidemiological investigation of prion disease occurrence in human and animal populations.
Main Results:
- Human prion diseases manifest sporadically (sCJD), genetically (gCJD), or through infection (vCJD from BSE).
- Iatrogenic (iCJD) and foodborne transmission routes are known, alongside historical cannibalistic transmission (kuru).
- Atypical prion diseases in sheep (Nor98 scrapie) and cattle (BSE-H, BSE-L) resemble human sporadic/genetic forms, suggesting potential animal equivalents.
Conclusions:
- Atypical prion diseases in animals may represent the animal counterparts of human sporadic or genetic prion diseases.
- The discovery of atypical prion diseases necessitates further investigation into their epidemiological links with human counterparts.
- Understanding these links is crucial for assessing and managing potential public health risks associated with prion diseases.
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