Prion Disease

BSE (bovine spongiform encephalopathy) also known as Prion Disease, is a disease many are still trying to understand as to why it happens. “Prion diseases or transmissible spongiform encephalopathies (TSEs) are a rare progressive neurodegenerative disorders that affect both human beings and animals” (Aguzzi, 2001). They are known to follow a long incubation period, along with substance like a spongiform changes related with loss of neuronal, and a lack to be able to show inflammatory response. The organism description, is the causative agents of TSEs are believed to be prions. “The term "prions" refers to abnormal, pathogenic agents that are transmissible and are able to induce abnormal folding of specific normal cellular proteins called prion proteins that are found and seen the most in the brain. The functions of these normal prion proteins are still not completely understood.”(Aguzzi, 2001) “The abnormal folding of the prion proteins leads to brain damage and the characteristic signs and symptoms of the disease. Prion diseases are usually rapidly progressive and always fatal. It is known that the likelihood of obtaining prion disease is by age. The older you are the more prone you are to it. Several mathematical models proposed over the past years predicted that the frequency of the disease would level off to zero around the turn of the century.”(Aguzzi, 2001) Most deaths occurred after being contaminated with this disease if usually one year after the onset of illness. A lot of research is still being done to help prevent this disease but no luck yet, but scientists do not understand what normal prion protein does but have figured out that “scientists believe that abnormal prion protein, which clumps together and accumulates in brain tissue” (DeArmond, 2006), is the likely cause of the brain damage that occurs in TSE diseases. Prions disease is also known as Prions “Protein,” This deadly disease was responsible for known epidemics of living organism it attacked humans and animals. Scrapie is a disease with the reputation of killing sheep and goats and has been recognizable for more than 200 years in Europe and other parts of the world. This disease may be more common in some countries more than others due to lack of knowledge of sanitation procedures. This infectious disease has many different names like for example Jakob Disease (CJD), Gersmann-Straussler Syndrome (GSS) GerstmannStraussler-Scheinker- Disease, and Kuro. Its known to attack the the brain and normally during this time it was attacking millions of people worldwide crippling them.” (Annual Reviews, Inc. 2001, p. 2). Its also known to attack all kinds of species especially felines, wild and domestic as well as cows, goats, sheep, bison, deer, elks it makes sense why wild cats get it they are meat eaters. There was never no information of how this disease was brought to native lands, this still remains a mystery. In the United Kingdom about 180,000 cattle became sick, what was known is that the cows owners were feeding these animals with other cow flesh! This was one of the most known cases however it was also spread in the mid 50's affecting people of Eastern Highlands of Papua New Guinea. Kuru or Prions Disease was being transmitted by eating other humans flesh in my opinion I think maybe it would have helped if they would have fried the meat even thou its probably out of our boundaries and norms today. This killer disease Scrapie caused its host go through excruciating symptoms like fatigue, insomnia, depression, weight loss, headaches, and in general malaise. It causes psychological problems in humans, changes in behavior, and people may start to lose the ability to speak or do any activities and may lead to mental retardation. It shuts down the body little by little, in a blink of an eye you can just see death occurring, seem familiar with All Timers but thats just my comparison. Anyway It also causes cerebellar ataxia, this means that you will lack the ability of muscle coordination, its scary but its pretty much eating the brain alive. If no proper assistance is given to the host, it will die under a six months, if you lived around old times era, you could just watch people die. (Annual Reviews, Inc. 2001, p. 2). “However there are different ways of getting the disease, you may acquire it by eating infected flesh or if you were eating flesh of any kind for instance be smart about it and cook it.