Prions Essay

1711 words - 7 pages

Report on Prions based on some articles. Excellent.PRIONSPrions have been a mistery for scientists from the day they where discovered. Prions act like viruses but they are not. Their structure and chemistry are unknown. They are believed to be proteins but that is yet to be completely proved.Prion stands for "proteinaceous infectious particles". Prions are known to cause many diseases involved with nervous systems like the brain. They are the ones that cause the well known " mad cow " disesase in Britain and "scrapie" for animals. For humans they are known to cause a rare disease in Papua New Guinea called Kuru ( or "laughing death") which striked only the cannibals in the Highlander tribes. Investigation led to the discovery of prions inside the of the victims brains that were eaten by the tribesmen that when they died, as a sign of respect their brains where eaten and the chain went on and on.The thing that makes prions so special is the fact that they lack the basic elements for reproduction, deoxyribonucleic acid and ribonucleic acid DNA and RNA respectively. This is what has given science a great deal of doubt as this would give the dogma of the beginning of live a radical turn.Prions have been in research for many years with experiments like the one done by Stanley B. Pruiser and his team of scientists at the School of Medicine of the University of California at San Francisco in which a study was carried out on mice to see if he was able to purify the scrapie agent ,another prion disease, in mice. But mice as humans took very long to develope the disease, for example Gerstmann-Straussler-Scheinker disease or fatal familial insomnia, which appear mostly on humans which have passed the age of forty and only in very rare cases before, so the experiment was changed to hamsters as these die faster because developed the disease earlier. One of the methods used for this purification process was using a centrifuge, that separates the component of a mixture according to their size and density. After a decade of experiments using the centrifuge method and other chemical methods, several discoveries were made : It was found out that the infectious particles were extremely heterogencous in size and density, the scrapie agent can be found in many molecular forms and the biological activity of the scrapie agent depends on a protein (PrP, called later when discovered it was a single molecular specie protein). This protein was found to be a glycoprotein (PrP): sugars are bounded to the amino acid, and it is half the size of hemoglobin. PrP is the protein, but we can also find today PrPc (for cellular) and PrPsc (for scrapie). Prp can be found in "steak" ( skeletal muscle) and also on the surface of lymphocytes present in milk, but there is no evidence that the ingestion of this things can cause disease in humans , but there is the still a risk.Amyloid hypothesis.Prions were found to form rods: long fibrils in brain tissues infected with scrapie and...

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