
A Prion Love Story (2013) - lobo_tuerto
https://www.newyorker.com/books/page-turner/a-prion-love-story
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owenversteeg
Stuff like this gives me a really nice, warm pride in the human race. As if
this is what we were born for: to get out there and do something incredibly
hard and life-threatening and on a monumental scale. I'm reminded of reading
the transcripts of Operation Epsilon [0]; the foremost scientists in the world
didn't think we could make an atomic bomb, but by concentrating incredible
amounts of incredible people we got there in a few short years. Impossible
things are frequently possible when a committed group of people set out to do
them.

In particular, as the article notes, prion research is damned hard, and they
had zero prior experience, and it's not the kind of thing you can put together
yourself in your backyard with eBay supplies. But love and pure force of will
pushed them forward and I don't think words could ever express how beautiful
that is.

[0] Operation Epsilon, in a nutshell, was when we took a bunch of German
nuclear scientists and put them in a bugged room and told them about Hiroshima
and listened to their reaction, in order to figure out how close the Germans
were to the bomb. I highly recommend reading the transcripts, they're
fascinating.

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mirimir
Woah. Fatal Familial Insomnia (F.F.I.). Caused by a dominant mutation, so ~50%
of children are affected. But typically not symptomatic until middle age.
Damn.

~~~
vecter
Just another example of how evolution selects for/against or doesn't select
for/against traits. If onset isn't until your 50s, it's plenty of time to
reproduce. Granted there might be reduced fitness because older people won't
be around as much to take care of children, but back during caveman days, very
few people probably lived to their 50s.

~~~
mirimir
Huh.

Indeed, searching "autosomal dominant late-onset" does yield quite a few, in
various species. Including autosomal dominant Alzheimer disease (ADAD).

~~~
kendallpark
Huntington's is also in this category. Although there may be anticipation
(onset gets earlier after each successive generation).

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tshanmu
Would be amazing to have a happy ending here. Such an inspiring story

~~~
ambivalence
Looks like there might be a happy end after all:

[http://www.prionalliance.org/2018/12/03/end-of-the-year-
upda...](http://www.prionalliance.org/2018/12/03/end-of-the-year-update/)

