According to the Center for Disease Control, "There has never been a case of nvCJD that did not have a history of exposure within a country where this cattle disease, BSE, was occurring" (CDC). Furthermore, the brains of nvCJD victims had proteins called prions which were very similar to those that were found in the brains of BSE infected cows. These prions were also slightly different than the ones that were found in the originalCJD(which affected mostly elderly people). As a result, it is very widely believed that nvCJD happened to be a rare disease which happened to pass from animal, to human. But how does nvCJD work and what does it to do people?.
The definitive answer as to how nvCJD works is not certain. However, there is a hypothesis which is widely believed to be true by the scientific community. It is believed that nvCJD is caused by a prion. "It was Stanley Prusiner of the University of California, San Francisco, who first discovered the nature of prions" (Henahan). Prions are nothing more than rogue or misshapen proteins. The problem is that these rogue proteins change the shape of normal proteins. According to Dr. Craig C. Freudenrich Ph.D.:.
A person ingests an abnormally-shaped prion from contaminated food. The abnormally-shaped prion gets absorbed into the bloodstream and crosses into the nervous system. The abnormal prion touches a normal prion and changes the normal prion's shape into an abnormal one, thereby destroying the normal prion's original function. Both abnormal prions then contact and change the shapes of other normal prions in the nerve cell. The nerve cell tries to get rid of the abnormal prions by clumping them together in small sacs that merge with its "stomach" (lysosome). Because the nerve cells cannot digest the abnormal prions, they accumulate in the lysosomes. The lysosomes grow and engorge the nerve cell, which eventually dies. When the cell dies, the abnormal prions are released to infect other cells.