Creutzfeldt-Jakob disease causes: Difference between revisions
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The CJD prion is dangerous because it promotes [[Protein folding|refolding]] of native proteins into the diseased state. The number of misfolded protein molecules will [[exponential growth|increase exponentially]], and the process leads to a large quantity of insoluble prions in affected [[cell (biology)|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, death within a few months, although a few patients have been known to live as long as two years. | The CJD prion is dangerous because it promotes [[Protein folding|refolding]] of native proteins into the diseased state. The number of misfolded protein molecules will [[exponential growth|increase exponentially]], and the process leads to a large quantity of insoluble prions in affected [[cell (biology)|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, death within a few months, although a few patients have been known to live as long as two years. | ||
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Revision as of 16:57, 20 November 2012
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Causes
Transmissible spongiform encephalopathy diseases are caused by prions. The diseases are thus sometimes called prion diseases. Other prion diseases includeGerstmann-Sträussler-Scheinker syndrome (GSS), fatal familial insomnia (FFI) and kuru in humans, as well as bovine spongiform encephalopathy (BSE) commonly known as mad cow disease, chronic wasting disease (CWD) in elk and deer, and scrapie in sheep.
The prion that is believed to cause Creutzfeldt-Jakob exhibits at least two stable conformations. One, the native state, is water-soluble and present in healthy cells. As of 2006, its biological function is unknown. The other conformational state is very poorly water-soluble and readily forms protein aggregates.
People can also acquire CJD genetically through a mutation of a gene (needs to be defined). This only occurs in 5-10% of all CJD cases.
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, death within a few months, although a few patients have been known to live as long as two years.