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The suspected cause of Creutzfeldt-Jakob Disease (the human form of "mad cow"), Bovine Spongiform Encephalopathy (the "cow" one), Scrapie (the sheep and goat form of "mad cow"), Chronic Wasting Disease (the form that affects ungulates like deer and elk), and Transmissible Spongiform Encephalopathy (in mink) is a protein molecule that has folded incorrectly, which is called a prion.

Basically, a protein molecule gets its function from both the molecular structure, and from the way the protein has folded. A misfolded or mutated protein molecule or prion can damage tissues attempting to use it, causing serious damage that is often irreparable, as is the case with BSE or Mad Cow Disease. It is thought that the cause of these misfolded proteins came from a cow or sheep that had a genetic mutation that caused this mutation in the protein molecule. Prions contain DNA, and it could have been the DNA in the affected animal that, upon creating the protein molecule, had one or more nucleotides that was not "read" properly by the tRNA replicating the strand of DNA prior to mitosis (a form of cell division). It is the structure of this prion that makes it so hard to destroy: it can withstand years in dead tissue, boiling and freezing. The only thing that will destroy prions is steam sterilization at a high temperature (132 degree Celsius [296 degree Fahrenheit]). It is also because of its structure that an animal's body does not produce any immune or inflammatory reaction against it, unlike pathogens like bacteria and viruses.

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9y ago

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