The Global Mail has an amazing story about how the last treks to find cases of kuru – a cannabalism-related brain disease – have been completed.
Prions are misfolded proteins that cause other proteins to take on the infectious misfolding. In the case of kuru it lead to shaking, paralysis, outbursts of laughing and a host of other neurological symptoms as the brain slowly degenerated.
No-one knew prions existed or could exist before kuru. But as the article makes clear, this ‘obscure disease’ of a remote tribe revolutionised our understanding of proteins and how infections could take place.
But the story is how it was discovered is more than just lab tests and the article is a brilliant retelling of the research.
Michael Alpers has been working on the research project since the 60s and recalls some of the episodes:
After each death, he says, “I would go and talk to the family again, and say, ‘Okay?’. They had participated in cutting up bodies in the past — so that was not an unusual activity for them. We had to clear a few people — particularly the women who were wailing. But some of the women stayed. The ones involved put on masks to protect the tissue and I had gloves.
“The father, or a close relative, would hold the head, and I would take the top of the skull off with a bone handsaw. It would take maybe 20 minutes… like cutting an avocado. I would go to particular parts of the brain… take out small cubes. My assistant would hold out the bottle that was relevant, take the lid off, and I’d pop it in.
“Then I’d take the whole brain out and put it in a bucket full of formalin and cotton wool so it wouldn’t be deformed, and put the lid on. All our samples would go into an insulated box. Then I put the skull cap back on, and sewed up. Then we said goodbye… gave everyone a hug, and took off. I did this five times. It was enough.”
It’s a wonderfully written, informative piece. A long and compelling read.