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posted by martyb on Wednesday September 07 2016, @08:57PM   Printer-friendly

NPR's "The Salt" column carries the grusome (but interesting) story of a medical mystery:

When researchers made their way to the highlands of Papua New Guinea in the 1950s, they found something disturbing. Among a tribe of about 11,000 people called the Fore, up to 200 people a year had been dying of an inexplicable illness. They called the disease kuru, which means "shivering" or "trembling."

Once symptoms set in, it was a swift demise. First, they'd have trouble walking, a sign that they were about to lose control over their limbs. They'd also lose control over their emotions, which is why people called it the "laughing death." Within a year, they couldn't get up off the floor, feed themselves or control their bodily functions.

Shirley Lindenbaum, a medical anthropologist with the City University of New York, who continues to write about the epidemic, knew it couldn't be genetic, because it affected women and children in the same social groups, but not in the same genetic groups. She also knew that it had started in villages in the north around the turn of the century, and then moved south over the decades.
...
Lindenbaum had a hunch about what was going on: In many villages, when a person died, they would be cooked and consumed. It was an act of love and grief. ... Women removed the brain, mixed it with ferns, and cooked it in tubes of bamboo. They fire-roasted and ate everything except the gall bladder. It was primarily adult women who did so, says Lindenbaum, because their bodies were thought to be capable of housing and taming the dangerous spirit that would accompany a dead body.

Finally, after urging from researchers like Lindenbaum, biologists came around to the idea that the strange disease stemmed from eating dead people.

The story goes on to explain that the disease wasn't spread by a virus or a bacterium, fungus, or parasite. It could survive being boiled into soup, and had no DNA. It was a totally new infectious agent.

It was a twisted protein called "prions," or "proteinaceous infectious particles", that could cause normal proteins in nerve cells to twist just like them, and slowly over long periods of time kill areas of nerve cells in the brain.

The story goes on to cover the similarity to Mad Cow Disease, a species jumping disease also caused by prions, and Chronic Wasting Disease that is affecting mule deer, white-tailed deer, elk, and moose in 21 states.

The CDC is working with public health authorities in Wyoming and Colorado to monitor hunters for signs of prion disease.

If the Zika doesn't get us, the Kuru probably will.


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  • (Score: 0) by Anonymous Coward on Thursday September 08 2016, @01:09AM

    by Anonymous Coward on Thursday September 08 2016, @01:09AM (#398947)

    Cleveland crystallographers claim pure prion protein:

    http://www.nature.com/nsmb/journal/v8/n9/full/nsb0901-770.html [nature.com]

  • (Score: 2) by MichaelDavidCrawford on Thursday September 08 2016, @01:15AM

    by MichaelDavidCrawford (2339) Subscriber Badge <mdcrawford@gmail.com> on Thursday September 08 2016, @01:15AM (#398950) Homepage Journal

    It's a fictional ninth state of water, a solid form whose melting point is greater than room temperature.

    It was previously unknown because it needs a seed crystal, but once you have just one such seed it needs to be handled with great care.

    Kurt Vonnegut explained Ice 9 to a Crystallographer that he met at a party. The Crystallographer grew visibly distressed then spent some time to himself thinking real hard.

    Finally he told Kurt that it was impossible.

    But now we have crystallized prions.

    --
    Yes I Have No Bananas. [gofundme.com]
    • (Score: 1) by khallow on Thursday September 08 2016, @02:22AM

      by khallow (3766) Subscriber Badge on Thursday September 08 2016, @02:22AM (#398967) Journal
      The oceans aren't made of ice 9. It's been several billion years since the world's creation. If ice 9 were possible, it would have happened by now.
      • (Score: 2) by MichaelDavidCrawford on Thursday September 08 2016, @02:41AM

        by MichaelDavidCrawford (2339) Subscriber Badge <mdcrawford@gmail.com> on Thursday September 08 2016, @02:41AM (#398979) Homepage Journal

        yeah both classical and quantum mechanical activities would lead to the spontaneous creation of a seed, but its crystal structure is quite complex.

        --
        Yes I Have No Bananas. [gofundme.com]
        • (Score: 2, Touché) by khallow on Thursday September 08 2016, @03:00AM

          by khallow (3766) Subscriber Badge on Thursday September 08 2016, @03:00AM (#398992) Journal
          Well, zero probability is extremely unlikely.

          You want more than just an extremely unlikely (but still nonzero) probability of a seed forming. The seed has to lead to further accumulation of ice 9. Basically, you're claiming that oceans of water has been supercooled for billions of years without dropping to a lower energy state. That's just bogus.
  • (Score: 0) by Anonymous Coward on Thursday September 08 2016, @04:18AM

    by Anonymous Coward on Thursday September 08 2016, @04:18AM (#399019)

    Another protein crystallographer was Nobel winner Herbert Hauptman -- from the press release at:
        http://www.nobelprize.org/nobel_prizes/chemistry/laureates/1985/press.html [nobelprize.org]

    When X-rays strike a crystal, they will be deflected only in certain definite directions, where the intensity of irradiation may be measured. To determine the arrangement of atoms in a crystal, however, it is not enough to know the direction and intensity. The "phase" of each ray so deflected must also be known. In special cases, it has been possible to solve this "phase problem" by making use of the fact that "heavy" atoms containing many electrons spread the X-rays more strongly than "light" atoms do. This property of heavy atoms is used both in "Patterson methodology", which has been very important in structural inorganic chemistry, and in "isomorph substition". The latter is used when determining the structure of giant molecules such as proteins. In this case the heavy atoms can be bound to the protein without its structure being appreciably altered. This however is not possible for the large number of compounds.