| You may be wondering what a prion is and why | | | | found that fit this pattern. These included scrapie |
| you should care. Well, your ears might perk up a | | | | (found in sheep), chronic wasting disease (found in |
| lot if you hear the words - 'Mad Cow Disease'. | | | | deer and elk), Creutzfeldt-Jakob disease and fatal |
| That disease is caused by a transmissible prion. | | | | familial insomnia in humans, and bovine spongiform |
| Prion related diseases have been recognized for | | | | encephalopathy in cattle (also known as mad cow |
| years. However, until recently we did not realize | | | | disease). |
| what caused them. | | | | What is not known is if there is a connection to |
| What we did know about them is that they | | | | prion disease and Alzheimer's disease. We have |
| caused dementia. People gradually lost their ability | | | | recognized several genetic mutations that can |
| to reason and remember things. We also noticed | | | | cause Alzheimer's disease and form prions in the |
| that there were typical changes in the brain when | | | | body of the person with the mutation. However, |
| looked at during autopsy. | | | | this does not account for the total number of |
| Prions became famous when Kuru was found | | | | those with Alzheimer's disease. |
| among the Fore people of New Guinea. In this | | | | One piece of evidence that is suspicious. It is |
| disease, people would get unsteady gait, tremors, | | | | known that people who habitually eat meat more |
| and slurred speech. The interesting thing was that | | | | than four times a week have three times as |
| only women and children seemed to get the | | | | much chance of getting Alzheimer's disease than |
| disease. | | | | vegetarians. However, at least some of this |
| It was finally noticed that when a person died, his | | | | statistic could be related to increasing |
| family would eat him as a way to honor the dead. | | | | arteriosclerosis (hardening of the arteries) in those |
| But only the women and children participated in | | | | that eat meat. |
| this practice. Scientists found that by stopping this | | | | If there is a prion that causes Alzheimer's disease |
| practice, the incidence of the disease suddenly | | | | that you can get from eating meat, why can't |
| plummeted. | | | | you easily prove or disprove it? Well, here is the |
| Further research in Kuru found that the cause | | | | problem. The currently know prions cause disease |
| was a protein - not a bacteria or virus. | | | | fast enough that you can put the prions into lab |
| Furthermore, there was no way to destroy this | | | | animals like white mice and they get the disease. |
| protein by filtering or cooking. Proteins are formed | | | | However, have you ever seen a mouse live as |
| as a long molecule that then folds over and over. | | | | long as someone who has Alzheimer's disease? Of |
| The shape of the folded molecule determines | | | | course not. |
| how it functions. | | | | We do know that prions come in strains that |
| It also determines what enzymes in the body are | | | | vary widely in the amount of time that it takes |
| needed to break it down when it is no longer | | | | to cause the disease. So, here is the big question. |
| needed. The prion works by causing a protein in | | | | What if there were a prion that caused disease in |
| the brain into the wrong shape. Therefore it does | | | | humans, but it took longer to cause problems |
| not work and the brain makes more and more of | | | | than the lifespan of the animal that it came from? |
| the chemical - trying to make enough of the | | | | For instance, if cows live 10 years but it takes 50 |
| protein to fill the need of the body. | | | | years for the disease to show up, you would |
| The real devastation of the disease though is that | | | | never - ever see the disease in cows even if |
| the resulting misshapen chemical does not have | | | | they carried the disease. The same goes for |
| enzymes in the body that can break it down! So | | | | chickens, sheep and pigs. You see the problem? |
| the protein builds up in large amounts that | | | | At this point, we do know that one fact - that |
| become toxic to brain cells and finally cause them | | | | you get only 1/3 as much dementia if you are a |
| to die. | | | | vegetarian. Until more is known about what is |
| Once scientists realized the cause and effect of | | | | really in fact safe to eat, vegetarians have a leg |
| this prion, several other known diseases were | | | | up on carnivores on this issue. |