h2g2 The Hitchhiker's Guide to the Galaxy: Earth Edition

Thanks, I'll try to do the background reading. Just indulge me in one more thing - what function do the normal prions serve in the body?

Vested interest section... I wrote a novel in 1997 - a thriller about mad cow disease, believe it or not. It never got published, but I might try to rework it if I can find the time. I never had the time to keep up with all of the science, though I have downloaded the entire British BSE inquiry report (all 16 volumes and 45 Mb).

Last time I looked they didn't know what the normal protein was there for. They bred mice without the gene for the prion and they were completely normal in every observable way.

Let me know when you get the book published. I'll get my copy ordered

I don't know how sound the science is, but in the BBC Wildlife article I've been reading (and referenced in the BSE theory thread here earlier) the researcher noted that prion proteins tend to be heavily clustered just under the skull and in particular behind the retina. His theory is that they are possibly acting as a defence mechanism against some agent that might creep in at those points and suggested ultra-violet light and oxidising agents that might be activated by the UV. What I've read is too sketchy to expand on with any expectation of accuracy but it's something to do with a tendency of prions to bind to copper or if unavailable, manganese. Too little copper and too much manganese means the prions can't function in their protective capacity and damage ensues.
As I said, the article isn't very detailed and there's obviously a lot missing from the above sequence of events but is there any chance this guy's on to something re the purpose of prions?

Yes! In verse...
I got my report back today,
On the front was a big fat "A",
So much info I'd cramed,
With a long epigram,
I think he was scared by the weight!

Yes! In verse...
I got my report back today,
On the front was a big fat "A",
So much info I'd cramed,
With a long epigram,
I think he was scared by the weight!

Stories are creeping out in the British press about BSE-infected cattle which have been killed in the foot-and-mouth fiasco.

They're saying that burning infected carcasses could release the "BSE agent" into the air. If prions are as indestructible as you say, then they may have a point. More important is the admission by a British government department that there are probably still a lot of cattle wandering about with BSE.

The rules say that cattle older than 30 months cannot be used for human food, but all that means is that the disease *probably* takes longer to develop in a noticeable form.

They're saying that the "BSE agent" can be detected in organs other than the brain. Sorry, I'd thought of that - it travels in the blood.

Are they saying that BSE/CJD infection is still available in British cattle?

I think so. Then again, I don't eat much beef or lamb and tend to be a bit paranoid when it come to foods that could kill me. BSE has not been reported in the US, but with republicans in change, we don't know what to believe.