Bovine spongiform encephalopathy

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By Kevin Pan

Overview

By Kevin Pan


Transmissible spongiform encephalopathies are known as prion diseases that are progressive neurological disorders. This group of diseases includes Kuru, which is a disease found among New Guinea natives, Gerstmann-Straussler-Scheinker disease and bovine spongiform encephalopathy. Bovine spongiform encephalopathy is a progressive neurological disorder in cows that arose in the 1980’s. Humans can be infected with this disease after eating products that have been tainted with this disease. While the exact method of transfer is still not understood, the disease is not believed to be a product of bacteria, viruses, parasites and fungus (bseinfo) It is believed that a misfolded protein known as the prion protein in cattle is the cause of transfer. Prion proteins are commonly found throughout the body, but they can be modified if there is an accumulation of the modified prion proteins Eventually, a high accumulation of these protein can lead to damaged and disease. A major issue with this protein is that it is difficult to kill as it is able to survive heat, UV light, ionizing radiation and normal sterilization techniques (USDA 2015). The prion is also partially resistant to Proteinase K, which is a type of amino acid breakdown. This leads scientists to believe that the prion is not destroyed in the gastrointestinal tract (Campbell 2006). It is believed that the variant Creutzfeldt-Jakob disease, which is the human form of BSE, is caused by BSE. However, studies have shown a similarity between prions of BSE and TSE, but not a directly correlation to support that theory2. Symptoms of CJD are dementia, memory loss, personality changes and hallucinations. Mad Cow Disease was first found in cattle in England in 1986 and thousands of cattle died due to the disease. Heavy restrictions were placed on cattle export throughout the world, with heavy restrictions occurring in Europe. The USDA implemented stringent measures, banning products from nations where BSE was found. Between 1985-95, it is believed that almost 1 million cattle have been infected in the UKK and about ¾ of those were fed to humans (Campbell 2006). This ban on UK beef was removed in May 2006. While the disease has appeared to be almost eradicated, it is still relevant to current research. Recent studies have shown a link between bovine prion disease and Alzheimer’s disease.



Figure 2 Structure of Prion Protein http://www.sciencemag.org/content/278/5336/245.full#F1



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