If you ever watched Boston Legal, Denny Crane played by actor William Shatner always blamed his “Mad Cow Disease” whenever he forgot something or blundered an important case. Mad Cow disease has similar symptoms to Alzheimer’s when people start out by having occasional lapses in memory. On the show Denny would do something out of the ordinary to keep people off track or unaware of his mild cognitive impairment. While this made the show entertaining, mad cow disease is no laughing matter. Luckily humans don’t get mad cow disease easily. Cows get mad cow disease by eating food that contained infectious parts of older dead cows. Cheap hamburger is made from old dairy cows comprised of thousands of body parts blown right off the bones of steers and cows. The older the cow the likelihood of mad cow or prion disease is more prevalent.
Prions are mysterious proteins that have an unknown function until they twist their shape and become a destructive force infecting other prions in their path. As these defective prions hit the brain they inflict devastation on their victim whether it be sheep, cows or people, they will literally lose their minds. Prions are connected to cannibalism, fatal familial insomnia and hamburgers.
The strange thing about prions to cause an infection you need to replicate and usually only proteins with nucleic acid can do that, but prions don’t. They have no nucleic acid or DNA. Scientist do not know what their primary function is and blocking prions doesn’t seem to affect the brain. All they do know is when one become infected it becomes a chain reaction and more likely death will result. How do prions get infected? If you ingest infected or malformed prions it will make healthy prions become infected, genetic mutation (bad DNA) as in fatal familial insomnia and randomly.
Infected prions will basically kill brain cells either all over or can pinpoint a particular area say the thalamus and over time will completely eliminate it. This process is slow and people don’t get sick or show any signs of mad cow until middle age. Fatal familial insomnia victims suffer from a stressful event before they show symptoms.
800,000 years ago in New Guinea the people practiced a ritual mortuary cannibalism which is the consumption of the dead as a way of honoring them to make themselves more powerful. They made the mistake of consuming people that were infected causing a prion plague and those that survived had it programmed into their genome. To this day some humans still have that program or signature in their genes. When mad cow came along it helped scientist understand the disease and limit the damage. Mad cow fortunately comes from a bovine prion protein and is different enough that it isn’t a very effective infector.
One way to deter mad cow disease is to test all the cows for mad cow, but as it stands the USDA doesn’t require testing as they try to protect foreign markets and prevent all beef producers from the expense. Of course the drug companies will benefit as they have two drugs that show results. The more we understand about prions and the failure of proteins to form properly will help people who have Alzheimer's, Parkinson's, Huntington's and Type II diabetes, let alone fatal familial insomnia.
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