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Andering_J_REDDSON
Andering_J_REDDSON
Creutzfeldt-Jakob Disease
Oct 22 2008, 10:20 PM EDT | Post edited: Oct 22 2008, 10:20 PM EDT
Creutzfeldt-Jakob disease is a very rare and incurable brain disease that is ultimately fatal very simular to bovine spongiform encephalopathy (commonly known as mad cow disease).
The CJD prion is dangerous because it promotes refolding of native proteins into the diseased state. The number of misfolded protein molecules will increase exponentially,and the process leads to a large quantity of insoluble prions in affected cells. This mass of misfolded proteins disrupts cell function and causes cell death. Mutations in the gene for the prion protein can cause a misfolding of the dominantly alpha helical regions into beta pleated sheets. This change in conformation disables the ability of the protein to undergo digestion. Once the prion is transmitted, the defective proteins invade the brain and are produced in a self-sustaining feedback loop, causing exponential spread of the prion, leading to death within a few months. 1  out of 3 found this valuable. Do you?    
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