I recently found out about the so-called prion diseases, which are incurable and fatal diseases that affect (a euphemism, the real word is destroy) the CNS and cause a rapid deterioration of mental and physical abilities.
There are many prion diseases, the two most famous are probably the mad cow disease (non-human mammals), and the Creutzfeldt–Jakob disease (CJD) (for human mammals). Both are 100% fatal, and *no one* is known to have survived longer than 2.5 years after a CJD diagnosis. That’s the kind of stuff you wouldn’t wish on your worst enemy.
Why are these diseases so deadly? I read that it has to do with abnormal proteins but that was way over my head.
EDIT : I have another question, can prion diseases be rightly called the most dangerous diseases known to man ?
Thanks;
In: Biology
Our bodies use proteins as building materials and for various functions in the body. They are crucial to our ability to live, and are fairly durable. A prion is a malformed protein that causes other proteins to become similarly malformed, spreading the problem and stopping their normal function.
For an analogy imagine you have an army (your body) fighting invaders (bacteria, etc). Your army men can attack the invaders (your immune system fighting back), and you can also use things like poison gas against them (antibiotics, etc). The poison gas you pick can be ones your army has gas masks against while the invaders do not.
But now imagine that your army encounters bolts laying around on the ground. These are standard machine or structural bolts, but they are flawed and brittle. Even worse when those bolts get near other bolts they make them brittle as well! A tank drives out into a field and comes back with a few brittle bolts. More of its bolts become brittle until the tank breaks, but it also spread the brittle bolt condition to other tanks. And to the workshop’s structural frame! This shop also services transport trucks which also use bolts.
The workshop collapses from bad bolts, and the base barracks. Some stretches of the perimeter fence also fall over. The transport trucks visit supply depots and contaminate the warehouses. From there more transport trucks spread the brittle bolts to the factories themselves, where production lines begin to break down. Your army is collapsing, it is doomed.
How do you fight this? Your army guys don’t recognize bolts as an enemy, and they can’t shoot them even if they could. Your poisons do nothing against the bolts, they aren’t even alive. Anything you can use that would destroy the bolts would also destroy whatever they are in and things around it as well. Once they are in your infrastructure the only way to get rid of them is to destroy it all, and if that infrastructure is critical? You are out of luck.
Ending the analogy, the only prions we know of infect the neurological system of the body. Once prions are in your brain there is nothing to be done, you are inevitably doomed. Luckily getting exposed to brain or nerve tissue is somewhat difficult.
Now to give you nightmares: I don’t know of any reason that other kinds of prions are not possible. Maybe a prion could exist that affects proteins in the skin, spreading via contact. Maybe you could have lung tissue impacted by prions spread by coughing. The proteins could remain dangerous indefinitely, only being deactivated by extreme temperatures exceeding 1000 Celsius. Cleaning chemicals and alcohol hand sanitizer does nothing. Infection is invariably fatal.
Sleep well.
Latest Answers