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953047.  Tue Nov 27, 2012 12:03 pm Reply with quote

A few people have written about Kuru and other prion diseases here and there over the years, so I thought I'd bring them all together in one thread and add in some stuff that I learned in university this year.

Frederick The Monk





Molly Cule


Kuru is a prion disease, part of a group of degenerative brain diseases that are unique in that they are infectious. Prion diseases are similar to other degenerative brain disorders in that the symptoms are caused by an accumulation of degradation-resistant proteins. Everybody produces prions - there are two forms of prion proteins: PrPc (prion protein cellular) and PrPsc (prion protein scrapie).

Prions are implicated in scrapie, CJD, vCJD (I learned recently that they aren't exactly the same thing), BSE and Fatal Familial Insomnia as well as Kuru.

More stuff to follow, but I've to cook dinner, so here's a PDF that formed part of my required reading.

Last edited by swot on Tue Nov 27, 2012 1:15 pm; edited 1 time in total

953052.  Tue Nov 27, 2012 12:53 pm Reply with quote

Chicken in the oven and veg prepped, it's time for more science.

Prions were discovered by Stanley B Prusiner, who named them after their attributes: proteinaceous infectious agents. The more astute among you will have noticed that this actually abbreviates to 'proin', but he changed in to prion, presumably because he didn't want to go down in history as the discoverer of something that sounds like groin. Prion diseases are also known as transmissible spongiform encephalopathies.

They cause a problem in their scrapie form because the abnormal shape is resistant to degradation, because the destruct site that attaches to proteases is not present. The normal cellular form is in the shape of an Alpha helix, whereas the scrapie form is in the much tougher Beta sheet. Collections of Beta sheets are the underlying cause of other neurodegenerative conditions such as Alzheimer's disease, Parkinson's disease, and Motor Neurone Disease.

Scrapie prions don't 'reproduce', in the manner of any other infectious agent, such as a bacterial infection, nor are they produced by the body. Occasionally they form spontaneously in the brain, or they are passed from patient to patient through consumption of infected brain or blood products, by transfusion of infected blood products or bone marrow, or by the use of infected surgical equipment (as well as being resistant to damage by proteases, prions are difficult to damage via normal cleaning methods, requiring much higher temperatures). Once in the new host, scrapie prions will 'recruit' the normal prions by causing them to misfold.

953055.  Tue Nov 27, 2012 1:14 pm Reply with quote

Here is the other PDF that I, and the rest of my class, were required to read.

In common with most prion diseases (with the notable exception of vCJD), Kuru has a late age of onset, due to the long incubation period. Kuru is usually not seen in people younger than 40 years old. It became prevalent, as everyone knows, in a Papuan tribe that practised cannibalism, and became rarer as the connection between eating people's brains and contracting their illness was made. My biology lecturer, at the very least, believes that it is possible that there will be a 'new wave' of CJD (or vCJD, I don't have my notes with me) patients around 20-30 years from now, as a result of all that beef we were eating before we knew it was bad for us in the early 90's.

That, along with the links in the OP and the wikipedia articles, is pretty much all I have on the subject for the moment. I'm sure I'll find more at some point (if you don't get there first), but there's a dinner to be faffed with.

966832.  Sat Jan 26, 2013 7:33 pm Reply with quote

I think if they were going to ask a kuru question, a good way to do it would be "What can make you die laughing?" Kuru can cause outbursts of laughter and is sometimes called laughing sickness or laughing death. Possible klaxons would be any mention of the Monty Python skit or any attempt at self-aggrandizement on the part of the panelists.

966921.  Sun Jan 27, 2013 6:51 am Reply with quote

Good idea :)

You could give honourable mention to the chap who died watching (IIRC) The Goodies.

Sadurian Mike
966926.  Sun Jan 27, 2013 6:53 am Reply with quote

The secret Black Pudding combat technique of 'Ecky Thump, to be exact.


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