Just recently, there was a study about what causes mad cow disease and Creutzfeldt-Jakob disease which are fatal brain disorders. These diseases are not caused by viruses or bacterium. Creutzfeldt-Jakob disease and mad cow disease are caused by an abnormal formation of a protein called a prion. Scientists and other studies are beginning to reveal that the prions help natural functions perform before they turn to the "dark side." Jiyan Ma, a molecular biochemist at OSU, and colleagues transformed a normal protein made by E. coli bacteria into a prion that had the properties of the infectious version. It starts out by forming clumps, declines being cut by enzymes, and changes other healthy proteins into the aberrant form. Jiyan Ma and his colleagues injected the prion into the brains of mice, which caused their brains to become spongy and full of holes, initiating telltale signs of prion disease. Their next step is to take a closer look at the system they used to make infectious prions to identify the molecular mechanisms behind the change.
In another experiment, researches in the U.S. and Austria used a prion protein also made by E. coli to contaminate hamsters with a transmissible brain disease. The diseases worsened very gradually, similar to how it progresses in humans, referring that hamsters could provide a useful animal model system.
While most research is about the disease-causing prion protein, Adriano Aguzzi of University Hospital of Zurich is looking at the healthy forms of those proteins in humans. He found that mice who laked those proteins showed a failure of the protective myelin sheath on their nerves. The main task now is to find out why this protein goes bad at times.
http://discovermagazine.com/2010/jun/04-untangling-prions-twisted-proteins-brains-into-sponge
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