
Deadly animal prion disease appears in Europe - etiam
http://www.nature.com/news/deadly-animal-prion-disease-appears-in-europe-1.19759
======
shmageggy
Prions are a crazy, crazy phenomenon. From wikipedia:

> _A protein as a standalone infectious agent stands in contrast to all other
> known infectious agents such as viruses, bacteria, fungi, and parasites, all
> of which contain nucleic acids (DNA, RNA, or both)_

In my layman's understanding, they're like this bizarre edge case in the way
proteins interact. Of all the myriad way a protien can fold, it happens to
find one that _induces the same malformation_ when it interacts with another
protein. To me, it almost seems like as much of a mathematical/geometrical
problem as a biological one. In any case, very interesting from the
perspective of emergent behavior in complex systems.

~~~
matthewmcg
Right--as I understand it, it's not that different than Google's "Meeting Room
Hardware Virus":

"I imagine it started with all of the adapters which were thrown around in
every meeting room. Everyone with a Macbook of some flavor needed a DVI to VGA
adaptor in order to use the projectors, so they were plentiful. Somehow,
someone probably damaged one of them and smooshed a couple of pins into places
where they should not have gone.

"Then, someone else forced this into their Mac. Perhaps two pins tried to go
into just the one socket. At any rate, it would now break the socket and get
it all out of whack. That socket, used with another adapter in another room,
would then break that adapter. This new broken adapter would then go on to
break even more Macbook DVI connectors.

Thus, we had a hardware virus." [1]

[1]
[https://rachelbythebay.com/w/2012/09/24/dvi/](https://rachelbythebay.com/w/2012/09/24/dvi/)

~~~
jotux
Similar things happen with PCMCIA cards. The connector design on PCMCIA cards
is just awful in that male pins are in the receptacle/PC. If you accidentally
clog one of the holes on the PCMCIA cards connector and plug it into a
receptacle it will bend the pin over.

~~~
tropo
Male pins on the card would fail just the same way, plus they'd also get more
damage from being exposed. The only upside would be easier repair and, in most
cases, cheaper replacement.

The correct fix would be pins with a better length/width ratio.

~~~
TheSpiceIsLife
Or no pins as at all but rather a PCB edge connector[1].

1\.
[https://en.wikipedia.org/wiki/Edge_connector](https://en.wikipedia.org/wiki/Edge_connector)

------
jcromartie
The most terrifying thing about prions is that they are infectious but not
_alive_ : they can't be "killed" in the normal sense by sterilization. The
proteins must literally be dissolved to neutralize them.

~~~
hodwik
I slept through biology class, could you explain how that's not also true for
virii, given that they are also not "alive"?

~~~
henrikschroder
> virii

Pet peeve of mine, but it looks like you slept through English class as well.
The plural of "virus" in English is "viruses". "virus" in latin is a mass word
and has no plural, like "sugar" and "air".

~~~
hodwik
Your perscriptivism here seems misguided then, because if virus is a mass word
we shouldn't be saying viruses either: just "virus". And anyway, if we
absolutely had to express a plurality of virus, it would be "vira".

Anyway, this is _hacker_ news.

For those of us who grew up in internet hacking communities of the early 90's
"virii" was the plural form in the lingua franca. As a descriptivist of great
habit, I will continue to use that form.

~~~
henrikschroder
I am actually a descriptivist myself, but the prescriptive approach usually
works best when trying to convince people how to write. Apparently not so in
this case.

How about this argument then: "virii" is f*ckin ugly? No? Darn. :-)

------
nxzero
Prions: Killer Zombies

"You Can't Kill What's Not Alive: Prions cannot be destroyed by boiling,
alcohol, acid, standard autoclaving methods, or radiation. In fact, infected
brains that have been sitting in formaldehyde for decades can still transmit
spongiform disease. Cooking your burger until it is well done will not destroy
the prions!"
[http://learn.genetics.utah.edu/content/molecules/prions/](http://learn.genetics.utah.edu/content/molecules/prions/)

"Eating human brains helped Papua New Guinea tribe resist disease, research
shows." [https://www.theguardian.com/science/2015/jun/10/brains-
helpe...](https://www.theguardian.com/science/2015/jun/10/brains-helped-papua-
new-guinea-tribe)

Original name for the prions was "trembling in fear" \- or Kuru:
[https://en.m.wikipedia.org/wiki/Kuru_(disease)](https://en.m.wikipedia.org/wiki/Kuru_\(disease\))

~~~
Mahn
So there's no cure, no vaccine, and you can't kill them. Terrifying. An
outbreak of this thing would have free pass to eradicate entire species,
including us.

~~~
Lawtonfogle
Oh, and the incubation time can be extremely long. A burger you ate 5 years
ago could've been infected but it could still be years before you begin
showing signs. I remember reading a bit about some connection between prions
and dementia in elderly individuals but I don't remember any of the details.

One sci-fi horror series had an episode where the plot was that some mass
produced chickens were getting ill because the feed was made from other
chickens and they eventually discovered that it involved a prion that was
dangerous to humans as well. This was discovered after massive numbers of
those chickens had been consumed in large quantities by society.

~~~
im3w1l
That's literally what happened with the mad cow disease scare if you swap cow
for chicken.

------
ckinnan
>>Although the disease is not known to be transmissible to humans

This is not quite correct, the disease is believed to be transmittable to
humans, but we're not in the practice of eating deer brains, so there are no
confirmed cases. All of the states with CWD have advisories on reducing the
risk with consumption. Eg:
[http://www.dgif.virginia.gov/wildlife/diseases/cwd/deer-
carc...](http://www.dgif.virginia.gov/wildlife/diseases/cwd/deer-carcass-
handling/)

Given that we don't really know how CWD is transmitted among animals it is
still pretty worrying.

------
martian
Prions are both beautiful and terrifying. One of my favorite science fiction
stories is Kim Stanley Robinson's "Aurora". He makes great use of the prion
concept, and its consequences for human life, in relation to space
exploration. Fascinating read if you're interested in the topic.

~~~
Kristine1975
Thanks for the recommendation! I started reading it, and it's well-written and
very interesting.

------
exhilaration
I recently learned that Chronic Wasting Disease was spreading through deer in
the United States, there's a map of its spread here:
[http://www.ct.gov/deep/cwp/view.asp?a=2700&q=323412](http://www.ct.gov/deep/cwp/view.asp?a=2700&q=323412)

Apparently it's been shown that spiker monkeys can contract it but it hasn't
(yet) been shown to be transmitted to humans.

~~~
mixmastamyk
How does that work, considering deer are vegetarians? Or are these farmed deer
being fed the last generation?

~~~
yorwba
From the article linked by GP:

How does CWD spread?

The method of CWD transmission is unknown, however there is strong evidence to
suggest that abnormally-shaped proteins called "prions" are responsible. The
agent responsible for this disease may spread directly through animal to
animal contact or indirectly through soil or other surface to animal contact.
It is thought that the most common mode of transmission from an infected
animal is via saliva and feces. A recent study confirms that CWD prions can be
shed into the enviroment in feces from animals showing no clinical signs of
the disease and can contaminate the soil, leading to infection in other
animals. CWD can be spread from region to region by the movement of captive
deer or through the improper disposal of a harvested deer transported from a
CWD infected area.

------
imaginenore
Prions are fucking scary. They survive all regular decontamination procedures
- alcohol, boiling, even autoclave. Patients had been infected through the
sterilized scalpels and endoscopes.

~~~
azazqadir
>>Patients had been infected through the sterilized scalpels and endoscopes.

For someone who had an endoscope recently, that's really scary.

~~~
zymhan
I mean, people were also at times infected with HIV and Hepatitis C from blood
transfusions, but we realized the risk and took steps to mitigate it.

------
Balgair
Since there are a lot of questions and very good discussions today, I thought
I would share a good resource for bio made for comp-sci people:
[https://www.cs.cmu.edu/~wcohen/GuideToBiology-
sampleChapter-...](https://www.cs.cmu.edu/~wcohen/GuideToBiology-
sampleChapter-release1.4.pdf)

If anyone knows any other good resources or intros, please share as well.

------
mrfusion
Could a prion have been the precursor to life? Seems simpler for a protien to
accidentally come into existence and start replicating than a string of rna?

Or am I misunderstanding?

~~~
debacle
It's likely that pre-RNA life and prions shared a lot of similarities.

------
technological
Anyone want to learn more about prion related disease Kuru .. should watch
this documentary

[https://www.youtube.com/watch?v=vw_tClcS6To](https://www.youtube.com/watch?v=vw_tClcS6To)

~~~
Grazester
I do remember reading about this disease and its spread through cannibalism

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jcfrei
Since when can we reliably test for prions? Could it be that those misfolded
proteins have been around forever?

~~~
FilterSweep
Possibly, but the modern age has considerably more vectors for exposure to
these misfolded proteins, and the last statement of what logfromblammo
said[0], these misfolding proteins don't always affect core processes of the
body.

[0]
[https://news.ycombinator.com/item?id=11534177](https://news.ycombinator.com/item?id=11534177)

------
alexholehouse
To be fair, this doesn't have to have originated via animal-to-animal
infection, but could be an example of sporadic CWD.

As far as I know, sporadic CWD is relatively uncommon, but given CWD is caused
by the PrP protein, and there are a number of known mutations in PrP which can
increase its likelihood of undergoing prion-conversion, I'd hope they're going
to sequence this animal's PrP gene to see it it shed's some light on the
etiology.

Irrespective, this could still mean that CWD is now endemic in Europe.

>>>

Updated for clarity and extra info/context (thanks pbhjpbhj!)

>>>

CWD: Chronic Wasting Disease (deer-based prion disease - main topic of
article)

PrP: The specific prion protein involved here. Note that (confusingly!) prions
are both a 'class' of proteins but also refers to a specific protein (PrP).

Prions (class) are proteins which can exist in one of two states. In their
soluble form they're happy-go-lucky proteins that are monomeric (i.e. exist as
a single unit). However, these soluble-form prions can undergo a
conformational change (re-arrange their shape) into a different conformation
(the infectious form). The infectious form of the prion can do two specific
things: 1) Aggregate (so all the previous soluble prion proteins get stuck
into a big wad of protein) 2): Catalyze the conversion of soluble-form prion
into the infectious form. Herein lies their infectivity - you get an
exponential growth in the number of proteins in the infectious state.

Prions (PrP) is a specific protein found in many higher-order multicellular
organisms that is the _SPECIFIC_ protein that causes a range of prion diseases
(Creutzfeldt-Jakob Disease (CJD), BSE [mad cow], CWD, Scrapie etc). There are
species barriers to these diseases, even though the proteins are pretty
similar (i.e. humans cannot catch CWD from deer, even though the PrP protein
misfolds in CWD and the same human version misfolds in CJD). These species
barriers are convenient (!!) but very poorly understood, which is somewhat
concerning.

Finally - it's worth point out prions aren't always bad. Fungi use them as a
mechanism to facilitate non-genetic heritability/diversity [1], and we're
increasingly finding examples of prion-like mechanisms that facilitate fast
and irreversible signalling in cells (e.g. in the inflammation response [2])

[1] True, H. L. & Lindquist, S. L. A yeast prion provides a mechanism for
genetic variation and phenotypic diversity. Nature 407, 477–483 (2000).

[2] Cai, X. et al. Prion-like polymerization underlies signal transduction in
antiviral immune defense and inflammasome activation. Cell 156, 1207–1222
(2014).

~~~
djsumdog
So humans can't get a prion disease from eating infected wild game?

Where does alzheimer's disease fit into this? Are there instances where plaque
build up is caused by protein misfolding, or are there other causes?

------
amorphid
Too bad DNA doesn't stick around more than a few million years [1]. It'd be
interesting to research the possibility of prions wiping out the dinosaurs
from Mad Herbivore Disease.

[1]
[https://en.wikipedia.org/wiki/Ancient_DNA](https://en.wikipedia.org/wiki/Ancient_DNA)

~~~
pavel_lishin
Why would preserved DNA help us figure this out?

~~~
djsumdog
Very true. It wouldn't at all would it? You'd need to have actual preserved
cells and protein from that era.

------
braderhart
Has anyone else read this, because it looks really promising?

[http://www.pbs.org/wgbh/nova/next/body/phage-alzheimers-
cure...](http://www.pbs.org/wgbh/nova/next/body/phage-alzheimers-cure/)

------
nsajko
Source: [http://www.vetinst.no/eng/Highlights/The-first-detection-
of-...](http://www.vetinst.no/eng/Highlights/The-first-detection-of-Chronic-
Wasting-Disease-CWD-in-Europe)

------
danielsiders
Also see the study on aerosol transmission of prions:

[https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3020930/](https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3020930/)

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tinix
All I can think of is this now:
[https://en.wikipedia.org/wiki/Starfish_wasting_disease](https://en.wikipedia.org/wiki/Starfish_wasting_disease)

------
mrkgnao
What does a normal reindeer brain look like?

~~~
gus_massa
I was thinking exactly the same. Or to be more precise, is it visible in the
image the damage caused by the illness?

I'm not feeling lucky:
[https://www.google.com/search?q=reindeer+brain&tbm=isch](https://www.google.com/search?q=reindeer+brain&tbm=isch)

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m_eiman
Off topic, kinda: is anyone else having problems with nature.com not
resolving? Firefox and Safari both fail to load it, but for some reason Chrome
manages to find the server. OSX, latest.

------
ioquatix
I, for one, welcome our new zombie reindeer overlords.

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chrramirez
For anyone who may want to know, prion diseases are sciencetific possible
causes of a Zombie Apocalypse. That's why this new called my attention.

~~~
StavrosK
By "zombie apocalypse", you mean slow-walking infected, craving brains and
infecting anyone they bite?

Or just, you know, a disease that kills you if you eat brains? Because that's
kind of the exact opposite of what zombies do.

~~~
lxmorj
A prion that infected people rapidly, through saliva-to-blood contact, and had
similar symptoms to rabies without the rapid death would be very similar to
traditional zombie lore.

The protein would probably also have to effect the olfactory glands, creating
an attraction to uninfected humans.

