Millenium Health Tips

Creutzfeldt-Jakob disease is a degenerative neurological disorder that is very rare and incurable.

Spongiform encephalopathy infectious disease is caused by prions, which are often referred to as prion diseases. Other prion diseases include Gerstmann-Sträussler syndrome-Scheinker (GSS), fatal familial insomnia and kuru in humans, as well as cattle spongiform encephalopathy commonly known as mad cow disease, chronic wasting disease (CWD) in deer, and scrapie in sheep.

Prions are believed to cause Creutzfeldt-Jakob disease at least 2 shows a stable conformations. Conformation in the native state, is water soluble and present in healthy cells. Until 2006, no known biological function. Other circumstances konformatif less water soluble and easily forms a protein aggregate.

People also can contract Creutzfeldt-Jakob disease through gene mutation (needs to be defined), which only occurs in 5-10% of all cases.

CJD prion is dangerous because it increases the protein from folding into a state of illness, which causes the increase of insoluble prions in affected cells. The mass of misfolded proteins disrupts cell function and cause death. Mutations in the gene for the prion protein folding can lead to mistakes mostly alpha-helical regions into beta pleated sheets. Changes 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 the feedback loop is supported himself, causing exponential spread of prions, death within a few months, although some people known to live for ever 2 years.

Stanley B. Prusiner was awarded the Nobel Prize in Physiology or Medicine in 1997 for the discovery prionnya. More than 1 decade, University neuropathologist Laura Manuelidis Yale dubious explanation for the disease. In January 2007 he and his colleagues published an article in the Proceedings of the National Academy of Sciences and reported that they found a similar virus particles (but so far without finding nucleic acids) in less than 10% strains of scrapie-infected cells and in mouse cell strains infected agent of CJD from humans.

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