Night of the Living Prions

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Proteins are very useful things. They are the little helpers to your body’s Santa Claus. They do pretty much everything that needs doing in your cells. They are great little workers.

Right up until they go wrong.

And the thing is – we have no idea how they could possibly go wrong. How could the little helpers turn against Santa?

One of the things that determines a protein’s function is how it’s folded up. What can happen is that a mis-folded protein makes its way into the body, and then takes a perfectly good protein and refolds it into a copy of itself. That, by the way, is the thing which is confusing the hell out of the medical world, because proteins have no genetic code of their own. They shouldn’t be able to make more of themselves – they are the end product, not the manufacturing plant. And so these misfolded proteins, called prions, spread, each one making copies which make more copies which make more copies…George Romero would be proud.

And what, you may be asking, is the worst that can happen when you misfold a few proteins? Eventually they get into your brain, and start turning your brain into a sponge.

Yes, a sponge.

This more commonly happens in animals, with the likes of BSE (Bovine spongiform encephalopathy) or scrapie. Which is why BSE is called ‘Mad Cow Disease’ – because it’s difficult to be a sane cow when your brain is turninng into a sponge.

It is, thankfully, fairly rare in humans. One of the very first times it was noticed in people, way back in 1958, was in a disease called Kuru.

This is a prion disease which was restricted to the Fore tribe in Papua New Guinea. In their culture when a person died, their remains were eaten to keep that person’s power within the tribe. Which makes as much sense as anything anyone does, and at least it doesn’t waste perfectly good meat.

Except what appears to have happened is that at some point some person in the tribe developed one of these misfolded proteins. And then their remains were eaten, introducing these prions into everyone else, and so it spread throughout the tribe. It always hit the women and children in the tribe more, because they got the less-nice remains to eat, such as the brain, where a lot of prions concentrate.

The prions can sit in the system for a long time before symptoms start to manifest – the average time for Kuru is around 14 years, but it can be as much as 40. But when it hits, you start to shake and giggle uncontrollably. Because your brain is, again, turning into a sponge.

Right now there are no cures for any prion diseases, but there are some promising pieces of research demonstrating how certain types of lichen, of all things, seem to be able to take out prions.

Unfortunately for the people on our train, however, their best hope is Michael and Evan…

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