Prions are a misfolded form of a normal protein. The normal occurring protein is PrPc and when it becomes misfolded it becomes a PrPsc, which is harmful. The misfolded proteins are hydrophobic and aggregate or clump together, causing amyloid plaques or fibers to form. (https://www.chem.wisc.edu/deptfiles/genchem/netorial/modules/biomolecules/modules/protein1/prot13.htm). This causes neuronal death, affecting the central nervous system and eventually leading to brain death …show more content…
When the prion turns the PrPc into a PrPsc, then it acts as a chaperone protein and converts more PrP into PrPsc in an irreversible act, forming amyloid plaques. This is how the disease is spread (https://prezi.com/wj8-zjs5dq3m/bse-101/).
Countries without regulations, can decrease the risk of transmitting the prion by making sure that their animal feed is free of ruminant, which is made from meat and bone of cows, sheep, goats and mink that would include brain or spinal tissue. Contaminated meet can contain the prions and are not easily destroyed (http://www.fda.gov/AnimalVeterinary/ResourcesforYou/AnimalHealthLiteracy/ucm136222.htm)
Animals should be monitored for symptoms of disease cattle with BSE may not show any signs of the disease for up to three to six years after they have been exposed to BSE prions. Symptoms may include, incoordination, difficulty standing, decreased milk production, nervous or aggressive behavior, abnormal posture and weight loss. These symptoms may progress for up to six months until the animal dies