"Bovine spongiform encephalopathy (BSE) is a cattle disease that is associated with central nervous disorders and is always fatal. The German translation (spongy brain disease of cattle) illustrates the effects on the brain of the diseased animals. Due to its transmissibility on the one hand and the very specific changes in the brain on the other, BSE is classified as a transmissible spongiform encephalopathy (TSE). This also includes human diseases such as Creutzfeldt-Jakob disease (CJD) and Gerstmann-Sträussler-Scheinker syndrome (GSS).
According to the so-called prion hypothesis, BSE is caused by the infectious, misfolded form of an endogenous protein, the prion protein. Although the final proof is still pending, scientists now assume that BSE can be transmitted to humans and causes a new variant of the fatal Creutzfeldt-Jakob disease (vCJD).
The BfR is working on the topic of BSE in the context of consumer health protection with the question of how transmission of the disease from animals to humans through food can be avoided. "