Prion diseases BSE scrapie Creutzfeldt-Jakob disease

Prion diseases

Wageningen Bioveterinary Research supports government and industry by carrying out diagnostics and scientific research and providing policy advice for the prevention and control of a large number of contagious animal diseases, including prion diseases.

BSE, scrapie and Creutzfeldt-Jakob

Prion diseases are transmissible spongiform encephalopathies (TSEs). These are a group of fatal neurodegenerative diseases that are caused by the misfolding of a certain protein that occurs normally – the prion protein or PrP. The protein then accumulates, in neural tissue in particular, and becomes infectious. These folded infectious particles are called prions. Examples of prion diseases are BSE in cows, scrapie in sheep en goats and Creutzfeldt-Jakob disease in humans.

Nervous system affected

Prion diseases normally have long incubation times. The symptoms are generally neurological because they affect the central nervous system. The transmission rate between species is generally the highest between individuals of the same species, but it may vary, also between individuals of the same species due to genetic differences in the prion protein and/or the circulating prion strain. At present diagnosis in living animals is generally not available and takes place post-mortem. The disease process is always fatal as there is no effective therapy for humans and animals.