Prion diseases BSE scrapie Creutzfeldt-Jakob disease

Prion diseases

Wageningen Bioveterinary Research provides diagnostic services, scientific research and policy advice to public and private sector organisations for the prevention and control of many infectious animal diseases, including prion diseases.

BSE, scrapie and Creutzfeldt-Jakob

Prion diseases are Transmissable Spongiform Encephalopathies (TSEs). These are fatal neurodegenerative diseases. They are caused by the misfolding of a naturally occurring protein known as the prion protein, or PrP. This leads to the protein accumulating, particularly in neural tissue, and becoming infectious. These misfolded infectious particles are known as prions. Examples of prion diseases are BSE in cows, Scrapie in sheep en goats and Creutzfeldt-Jakob disease in humans.

Damage to the nervous system

Prion diseases generally have a long incubation period. Symptoms are mainly neurological as damage is caused to the nervous system. Transmission occurs most easily between individuals within the same species. However, the ease of transmission can vary, even between individuals of the same species, as a result of hereditary differences in the prion protein and/or the prion strain being circulated. At the present, diagnosis in living animals is generally not available and takes place post-mortem. The absence of effective treatments means that the disease is always fatal in both humans and animals.