Artificial Prion Created

jabberjaw writes "Nature is reporting that researchers at the University of California San Diego have created a synthetic prion which, when injected into mice will bring about symptoms similar to those displayed by cattle suffering from bovine spongiform encephalopathy, aka mad cow disease. The researchers first crafted healthy prion proteins using bacteria. They then shook these proteins until they resembled the tangled structure of an unhealthy prion. Afterwords, these prions were injected into the brains of mice who fell ill two years later. Perhaps someone who is more familiar with this field of research would care to fill us in on the details as the article was rather light."

Comments

this is truely scary

[airbie]

because prions are more basic and fundamental than even germs/viruses. most modern methods of treating diseases and fighting virus involve disrupting the replication process of the virus/germs, usually by the means of inhibiting certain proteins. however prions themselves are malformed proteins that malform other good proteins. this mechanism is quite hard to stop because it is so simple, there is no complicated repoduction chain to disrupt like a virus. there is only one way to stop this chain, which is to basically burn the protein to a crisp.

Re:whoo hoo?

[MadBiologist]

That's most likely... by creating a model disease in a biological system, different drugs can be tried out on it to test efficacy.

There are all sorts of protocols like this already... ie, EAE (Experimental Allergic EncephaloMyelitis) where they give mice... um... artificial MS, then test drug canidates out on them to see what the effect is. It's not very nice to watch.

That's why I'm glad that I'm in molecular studies. However, it's done to help people with these diseases, and animal reseach is really the only way to conduct some of these tests.

Peace...

Re:The TRUE source of Mad Cow Disease?

[value_added]

Maybe if we'd consider the crazy idea that in nature, herbivores don't eat other herbivores, but when fed a regular diet made up of their friends and family, weird shit might happen?

Ah, the mandatory fscking stupid conspiracy theory

[Moraelin]

Wasn't feeling like /. without some idiotic conspiracy theory. And hey, look, it's the old favourite: those evil pharma corporations are all refusing to develop a cure.

Never mind that:

1. There _is_ money in a cure. If you had a cure for, say, Cancer and a 20 year patent on it, you could sell it for any sky-high amount of money you'd want to. It's the perfect extortion scheme. You pay up or you die a slow painful death.

2. Lo and behold, they did make cures and vaccines for a metric buttload of diseases.

3. Most importantly: there are doctors, pharmacists, managers at pharmaceuticals corporations, etc, who die of Cancer every year. Or have a bad case of diabetes. Or whose _child_ is dying of some disease.

Now you're telling me no less than they'll rather patiently wait for their own death -- or the death of those they love -- rather than break the conspiracy oath and divulge the cure. Doesn't that strike you as a completely retarded idea? If _you_ could make a cure, and you'll _die_ if you don't, wouldn't you just make the stupid cure already?

4. We're talking millions of doctors, pharmacists and researchers world wide. Some in countries where they don't even have private enterprise as you know it. (E.g., until recently the USSR and to some extent still China.) Or where it's not even easy to keep in touch with a western conspiracy. (E.g., the USSR block was pretty much isolated and guarded by a paranoid secret police.) And which invested hundreds of billions in researchs. (E.g., in developping their own nuclear weapons, sattellites, chemical weapons, biological weapons, ICBMs, etc.)

Yet you'd want me to believe that _all_ those are part of the same global conspiracy to keep some diseases untreated.

You know what? There's a medical name for that. It's called "Paranoia".

Re:whoo hoo?

[Sgt York]

Does anyone else read these things critically?

Well, seeing as that it's in Science, it probably was reviewed. RML is a purifed prion preparation (made by Rocky Mountain Labs), and is a common positive control in prion studies. To the grandparent: the scientists that still dismiss prion disease are few and far between. The majority of the scientific community accepts the prion mechanism of transmission now. This was not the case a few years ago, but it's an accepted mechanism now.

All that said, I do think it's a bit of a stretch to call these "synthetic prions". I only skimmed the paper so far, but as far as I can tell, they state that these are only infectious in mice that already overexpress an aberrant protein (16 fold!) in the CNS.

The big thing that gets me is the lack of controls. This is a Science paper; where is the CD1 mouse infected with the 'synthetic prion'? My guess is that it didn't get disease, so they excluded the data. Fig 1C starts to show this a little, but still lacks proper controls. Here, they show that brain homogenate from 9949 mice hit with seeded protein can induce disease in normal (FVB) mice. They still don't do complete controls, though. Where is the homogenate + CD1 mouse? The FVB + vehicle mouse? Heck, where are the loading controls? Come on, a Science paper without loading controls???? It's not exactly the kind of thing that belongs in "unpublished data".

The positive side is that they seem to have confirmed the role of beta-rich regions in prion disease, and the importance of crystal/amyloid formation (which has been downplayed recently due to gross pathologic findings; this suggests that micro-plaques will also cause disease). Hammering out the structural domains responsible for disease is an important step.