Spongiform

Dialogues frontispieceI wasn’t a very good university student. Oh, I obediently followed all the stereotyping rules — long hair, scruffy clothes, indolence, poor diet, insufficient personal hygene –but I wasn’t very good at the job itself. I didn’t study, missed tutorials and lectures, and generally dicked about. I didn’t get a first class degree.

Instead of doing what I was supposed to be doing, I’d frequently browse around the science library, just reading whatever took my interest. One thing I distinctly remember was finding a copy, right there on the shelves, of Galileo’s Dialogues of 1632 (or rather, the first English translation, by Salusbury in 1661). That was the one the Pope didn’t like.

There was also a stack of Scientific American magazines, going back to the Nineteenth Century (it was first published in 1845). (Let me point out that I went to university in the Twentieth Century. Let’s be quite clear on that.) It was in a relatively current issue though, that I first read about transmissable spongiform encephalopathies.

It was probably the first publication in a nonspecialist medium by Stanley B. Prusiner where he described the various diseases and explained his “prion” hypothesis. He got a Nobel Prize for his work, almost 20 years later, in 1997. I remember being particularly fascinated by one of the diseases which Prusiner had studied, Kuru, the laughing sickness. It’s almost eradicated now, but it was common among the Fore tribes of New Guinea, from about the 1950s onwards.

Kuru causes dementia and death, and is transmissible through close contact with infected tissue, especially nerves and brains. The Fore were cannibals, ritually eating their deceased ancestors. They also liked to rub brain tissue into their skin.

But the real health problem concerning Prusiner at the time was Mad Cow Disease, or BSE, Bovine Spongiform Encephalopathy. He noticed the similarities to Kuru, and began to suspect a similar transmission mechanism. That is, cannibal cows. Sure enough, he found that it was common (at the time of his article, and for ten years afterwards) to feed rendered meat products to cattle.

What do you think of this BSE then? Doesn't bother me - I'm a helicopter!If Prusiner was right, and it’s now almost certain he was, BSE, and the human and other variants, are extremely unusual diseases in that they are not spread by bacteria or viruses or any other living thing. You catch a transmissible SE by ingesting a “bad” protein molecule, one which is chemically correct, but just bent the wrong way. In your brain, the faulty molecule becomes a template, causing your own molecules to convert to the wrong shape, and they in turn convert others, and so on. The incorrectly-formed molecules don’t work the way they should, and slowly your brain becomes a holey mush. Since the infectious molecules (Prusiner called them “prions”) aren’t alive, they can’t be killed, so normal sterilisation doesn’t harm them.

I learned lots at university. Just not the stuff they ask you in exams.

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