Bovine Spongiform Encephalopathy :: BSE Prion Mad Cow Disease

Bovine Spongiform Encephalopathy (BSE) BSE is a disease found in cows that contaminates the brain. It kills many cattle each year because there is no known cure. Humans can be infected by a disease called Creutzfeldt - Jakob disease and sheep can be infected with a disease called scrapie. There is no known treatment for any of the Transmissible Spongiform Encephalopathy, including CJD. In order to prevent this disease, the beef is inspected before coming into the United States. Without antibodies, a microscope is the only other way to confirm the cow as being infected. Bovine Spongiform Encephalopathy, other wise known as mad cow disease is â€Å"an infectious degenerative brain disease occurring in cattle† (www.dictionary.com). Bovine Spongiform Encephalopathy is one of many Transmissible Spongiform Encephalopathy’s (TSE) in animals. Humans can not be infected with BSE but if they are exposed to contaminated meat, then they are at risk of being infected with Creutzfeldt - Jakob disease (CJD). CJD is very rare and as well as BSE, cannot be treated because there has been no cure found. Figure one shows the death rate of humans infected with CJD. (BSEInfo.org July 27, 2006) Figure one BSE mainly occurs in the brain but experiments have been conducted and found it to be â€Å"in the spinal cord, and in the retina of the eye. The BSE agent may also be present in the small intestine, tonsil, bone marrow, and dorsal root ganglia (lying along the vertebral column)† (US Food and Drug Association, September 24, 2004). Once the cow is infected, the brain tissue takes on a sponge-like appearance under a microscope. This is how the disease got the name Spongiform. (Mad Cow Disease, Andrew Simmons) BSE is spread throughout the brain by important proteins called prions. The prion folds itself incorrectly which leads other prions that encounter the false protein to fold as well. Spreading in the brain then occurs. Figure two shows how the brain would look when mad cow disease is present. These infected prions are extremely difficult to destroy, resulting in no possible cure. Figure Two There are several ways to tell of a cow is infected with BSE. An adult cow may take from two to eight years to first show signs of being infected but others can take a short time. The symptoms for infected cows are â€Å"anxiety, nervousness, and initial hyperactivity followed by lethargy† (Ratzan 10). Also cows experience pain in their horns, horn sockets and ears when they are infected.