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Sunday, August 14, 2011

Prions, How Do They Work?


Also, what the hell are they? To be honest, before researching this blog entry, my image of a prion consisted of a small ball with a happy face on it that gave you mad-cow (as it turns out, I wasn’t too far off).
Happy smiley face
According to American Heritage Dictionary, a prion is “a microscopic protein particle similar to a virus but lacking nucleic acid, possibly the infectious agent responsible for scrapie and other degenerative diseases of the nervous system.” Thanks, the internet! Now I know exactly what a prion is! I am so happy about that.

But what does a prion do? For this answer, I turn to a more serious-faced resource, the Centers for Disease Control and Prevention (remember the CDC? It’s been mentioned in movies). The CDC doesn’t like prions. They say all sorts of bad things about them. This is probably because prions cause horrible, debilitating diseases that end in agonizing death. Here’s what they have to say about the matter:

A prion is an abnormal, transmissible agent that is able to induce abnormal folding of normal cellular prion proteins in the brain, leading to brain damage and the characteristic signs and symptoms of the disease. Prion diseases are usually rapidly progressive and always fatal.

That sounds bad. It sounds bad because it is bad. Prions basically eat our brains. Here is the CDC’s list of diseases for which prions are thought to be responsible:

Creutzfeldt-Jakob Disease (CJD)
Variant Creutzfeldt-Jakob Disease (vCJD)
Gerstmann-Straussler-Scheinker Syndrome
Fatal Familial Insomnia
Kuru
Bovine Spongiform Encephalopathy (BSE)
Chronic Wasting Disease (CWD)
Scrapie
Transmissible mink encephalopathy
Feline spongiform encephalopathy
Ungulate spongiform encephalopathy

So, my original idea of a prion being a smiling ball that kills has been revised. A prion is an evil squiggly line that kills. Here is an artistic rendering using 3-D technology: 

BOVINE PRION PROTEIN 1dx0 asym r 500

2 comments:

  1. "Fatal Familial Insomnia"? What on EARTH does that mean? Your family keeps you awake until you spontaneously die from exhaustion? Where do they come up with these names, these so-called "scientists"?

    On another note, it's a shame that Prions really aren't just small balls with happy faces on them (sans the mad cow bit). I'd really rather they were.

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  2. I'm not really sure what they are talking about with that one. Maybe you go to sleep and never wake up...and then die.

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