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Fear and Trembling: Prion diseases on Twitter

A public lecture introduced me to the terrifying world of mad cow disease and kuru. But to really understand what there was to fear, I had to dig deeper.


  1. You have probably heard this story before. Even if you don't immediately recognize the words "prion" or "Kuru", the history has seeped into popular culture, like a horrifying fairy tale, or an urban legend that just happens to be true. Once, there was a tribe in New Guinea that ate the dead. It wasn't the kind of fakey cannibalism you see in the movies, with hunters rushing out to spear people for sustenance. Instead, it was about respecting your elders. When a member of your family died, you ate them -- you took a part of them into yourself. And that included the brain. 
  2. But over time, these people found themselves plagued with a terrible illness. Children and perfectly healthy adults, usually women, would suddenly begin to lose control of their limbs. They would jerk and shudder. Within weeks, they wouldn't be able to stand up at all. And then they died. 
  3. Everybody who had those symptoms died. 
  4. Eventually, Western scientists would learn the awful truth. When the people from New Guinea ate their ancestors they were also eating a disease. It attacked their brains --riddling the tissue with holes. The New Guineans, the Fore people, called the disease Kuru. In their language it meant "trembling" or "fear". 
  5. That story is true. Mostly. It happened in the 1950s and 1960s. Today, we know a little bit more about the disease, kuru. We know it's not caused by a virus or a bacterium or a fungus. We know it's related to other brain-damaging diseases, including Creutzfeldt-Jakob disease, which turns healthy adults senile and kills them within a year of the onset of symptoms; scrapie, which affects sheep; and the dreaded bovine spongiform encephalopathy -- mad cow disease. 
  6. Tying all these diseases together is a scary little something called a prion. On August 16th, I attended a lecture by Jay Ingram, a Canadian journalist who has written a book about prion diseases, called Fatal Flaws. The lecture taught me a lot about prions, but it also taught me about some of the flaws inherent in trying to live-tweet a lecture as I'm listening to it. 
  7. Public lectures are fascinating introductions to a subject. Twitter is a great way to share information with people who can't be in the live audience. But they are both, by necessity, short summaries of much deeper stories. When you combine the two, it's easy to end up with a collection of snappy ideas, rather than a deep, context-laden narrative. And that can be the difference between education and sensationalism.
    The tweets I wrote during Jay Ingram's lecture got a lot of attention. But as I looked at the questions and criticisms some of my readers had -- and as I started to read Ingram's actual book -- I realized that the missing context of Twitter might be leading people to conclusions that weren't correct. That's why I'm writing this up as a Storify. I want to take the disconnected ideas and fit them into a bigger whole. I also want to give you some things to think about the next time that I (or anybody else) live tweet a lecture.
  8. I tried to make it clear that I was quoting Ingram here. And, in general, most tweets from a lecture are quotes. But this is one of the places where it becomes difficult to understand the context. Am I, as the tweeter, telling you what I think? Am I simply relaying what was said by someone else? I can tell you what a speaker says, or I can tell you whether what that speaker is saying matches up with the bigger picture of evidence and opinion. The problem is that a live tweet of a public lecture can be a mixture of both. And knowing which perspective you're reading matters. Sometimes, it helps to ask before you re-tweet. 
  9. That's not to say that Ingram is incorrect in this quote. Prions do represent a revolution in how we think about biology. That's because prions are simply misfolded proteins. 
  10. Proteins are everywhere. Your body is built out of them. There are proteins in your cells that make the cells function. There are proteins in your hair, your skin, your muscles. Proteins control your metabolism, allowing you to turn a sandwich into energy. There are proteins in your brain. 
  11. Every protein is made up of amino acids, the little molecular building blocks of biochemistry. In his talk, Jay Ingram had a really nice model that will help you visualize this stuff. Imagine a pearl necklace. 
  12. Natural Grey Freshwater Pearl Necklace ~ Accented with Rhodolite Garnets ~ Pearl Drop Necklace
    Natural Grey Freshwater Pearl Necklace ~ Accented with Rhodolite Garnets ~ Pearl Drop Necklace
  13. Now, imagine that necklace twisted and turned, folded back on itself in a complex pattern. 
  14. Natural Grey Freshwater Pearl Necklace ~ Accented with Rhodolite Garnets ~ Pearl Drop Necklace
    Natural Grey Freshwater Pearl Necklace ~ Accented with Rhodolite Garnets ~ Pearl Drop Necklace
  15. That's what you should be thinking of when you think about proteins. At its most basic, a protein is just a chain of amino acids. But it gets its power -- an individual protein gains specific skills and tools -- because of how that chain is folded. In some ways, that's a great system. It allows you to do more things with the same set of tools, as if your screwdriver could suddenly become a hammer.
  16. The catch: The same protein can act in very different ways, depending on how it's folded.