The infectious agent in BSE is believe to be a specific type of misfiled protein called a prion. BSE is a type of TSE ( Transmissible spongiform encepahtopathy). TSEs can arise in animals that carry an allele which causes a previously normal protein to contort themselves from an alpha helical structure to a beta pleated sheet. This beta pleated sheet is the disease causing shape of the protein. Transmission mainly occurs when healthy animals come in contact with infected tissue from infected animals.
In the brain,