France issues moratorium on prion research after fatal brain disease strikes • Hacker News Board

Re: France issues moratorium on prion research after fatal brain disease strikes

#211

Post by nlitened » Wed, Jul 28, 2021, 4:10 PM UTC

fsloth wrote: post #48

Could a cure be to stop proteosynthesis of the prion-protein? https://blogs.scientificamerican.com/artful-amoeba/prions-ar... "This protein, in its healthy, properly-folded state, is, if not trivial, relatively unimportant. Its complete loss is certainly not catastrophic." I have no idea how to block proteosynthesis of a single protein in humans, maybe more biologically attuned HN:ners can comment what it would mean…

> "This protein, in its healthy, properly-folded state, is, if not trivial, relatively unimportant. Its complete loss is certainly not catastrophic."

If I at my age know anything about how real world works, absence of evidence is not an evidence of absence, and I can bet that this will turn out to be complete bullshit, and it will get known in the future that this protein is important.

Re: France issues moratorium on prion research after fatal brain disease strikes

#212

Post by ricksunny » Wed, Jul 28, 2021, 4:12 PM UTC

jtbayly wrote: post #93

dekhn wrote: post #56

Prions are weird. I was at UCSF during the time they went from a fringe theory to an established fact and a nobel prize. The professor who studied them (Prusiner) had to overcome tons of resistance and the experiments were heroic (often waiting months for mice to die). It's really funny that everybody resisted it until somebody mentioned that there was a plausible physical mechanism of refolding catalysis.

Makes complete sense that there was resistance until a viable mechanism was proposed. Otherwise it just sounds like voodoo.

If ancient Romans could have shown correlation & causation between lead pipes & drinking vessels to poor health outcomes, they should have ceased using lead for these purposes even while lacking the capability of understanding the underlying mechanism. There's no voodoo in that.

Re: France issues moratorium on prion research after fatal brain disease strikes

#213

Post by jjk166 » Wed, Jul 28, 2021, 4:18 PM UTC

fallingknife wrote: post #69

mschuster91 wrote: post #62

Earlier quoted context omitted.

> Why does every lab have to be shut down for 3 months? Auditing everyone and their processes takes a while.

Why would you audit processes when the allegation is that someone got infected because she wasn't following the processes?

Someone not following a process is a failure of the process. The process should include proper training and regular oversight to ensure that the process is being followed - an audit will reveal if that training and oversight is in place. If you trace any accident back far enough, you'll eventually get to a point where it could've been prevented with better planning.

Re: France issues moratorium on prion research after fatal brain disease strikes

#214

Post by persedes » Wed, Jul 28, 2021, 4:18 PM UTC

nebopolis wrote: post #162

resoluteteeth wrote: post #75

Earlier quoted context omitted.

Is there some specific reason to think that prions are harder to denature than other proteins? It seems like mad cow disease could also spread simply because meat is not normally cooked to ensure that 100% of the proteins in it are denatured.

Prions are essentially already denatured/unfolded. They are a more stable state of a normal protein that has biological function, and they interact with the "correctly" folded proteins to cause them to denature into additional prions. Often denaturing a prion means actually breaking apart the protein into fragments or amino acids, because it is already at the most stable/denatured state. This is also why they are so…

In addition prions seem to contain disulfide bonds, which tend to make proteins even more stable. (RNAse for example has them and is notoriously hard to get rid off unless you treat it with essentially H2S).

I haven't found a source for that, but according to my bio teacher mad cow disease started, because they decided to lower the temperature to pretreat animal waste (such as bones and brain matter...) which then ended up in what cows get fed with. Wikipedia mentioned nowadays they simply leave out the bones and brain from the food.

Re: France issues moratorium on prion research after fatal brain disease strikes

#215

Post by [deleted] » Wed, Jul 28, 2021, 4:21 PM UTC

M2Ys4U wrote: post #108

rs999gti wrote: post #73

Earlier quoted context omitted.

> Basically, you get infected, you die. Avoid cannibalism and you should be fine. https://www.npr.org/sections/thesalt/2016/09/06/482952588/wh...

Avoid eating British beef in the 1980s/1990s, too. Apparently 1 in 2,000 people in the UK may be infected.[0] We still don't know if there's going to be a second wave of disease from infections from that time period due to genetic factors influencing incubation of the disease. This is why people who were in the UK for longer than a few months during the 80s and 90s aren't allowed to give blood in various places aroun…

[deleted]

Re: France issues moratorium on prion research after fatal brain disease strikes

#216

Post by neolog » Wed, Jul 28, 2021, 4:22 PM UTC

newsbinator wrote: post #167

opwieurposiu wrote: post #130

Apparently some humans are running a patch that fixes this prion bug. How do we get this merged into mainline? https://www.nature.com/articles/nature.2015.17725

That's a great question. I would really love an answer. > CRISPR-Cas9-Based Knockout of the Prion Protein and Its Effect on the Proteome https://journals.plos.org/plosone/article?id=10.1371/journal...

That's a nice mechanism but wouldn't we all have to rebase from lucy?

Re: France issues moratorium on prion research after fatal brain disease strikes

#217

Post by neolog » Wed, Jul 28, 2021, 4:24 PM UTC

harles wrote: post #88

AbrahamParangi wrote: post #9

The study of rare, contagious diseases seems likely enough to result in an eventual outbreak that it should be treated like enriching uranium. Which is to say, with great caution- and not like regular science but rather like the kind of science with potentially very negative externalities.

Known prions can’t cause an outbreak without extraordinary methods of spread, such as cannibalism.

CJD is heritable.

Re: France issues moratorium on prion research after fatal brain disease strikes

#218

Post by agumonkey » Wed, Jul 28, 2021, 4:24 PM UTC

dekhn wrote: post #56

Prions are weird. I was at UCSF during the time they went from a fringe theory to an established fact and a nobel prize. The professor who studied them (Prusiner) had to overcome tons of resistance and the experiments were heroic (often waiting months for mice to die). It's really funny that everybody resisted it until somebody mentioned that there was a plausible physical mechanism of refolding catalysis.

any article about the replication mecanism ? not too short, not too detailed (if possible) thanks

Re: France issues moratorium on prion research after fatal brain disease strikes

#219

Post by subroutine » Wed, Jul 28, 2021, 4:34 PM UTC

hallway_monitor wrote: post #104

imglorp wrote: post #6

> She had not been trained in handling dangerous prions or responding to accidents and did not wear both metal mesh and surgical gloves, as she was supposed to, says Julien Bensimhon, the family’s lawyer. The thumb should have been soaked in a bleach solution immediately, which did not happen, Bensimhon adds. Does bleach even affect proteins? Isn't that protocol for an organism you're afraid of?

I was thinking what if she amputated her thumb immediately? It seems like an extreme reaction, but could it have prevented her infection and death?

That'd be a bold move, considering (1) it may not have helped, and (2) it is possible the thumb incident wasn't how she got infected. It's possible she got infected some other random day in the lab. Maybe she rubbed her eye while wearing a contaminated glove, or breathed in brain flecks through a poor fitting N95, while slicing a particularly dry cryosample.

Re: France issues moratorium on prion research after fatal brain disease strikes

#220

Post by heavenlyblue » Wed, Jul 28, 2021, 4:37 PM UTC

dangerbird2 wrote: post #206

hef19898 wrote: post #149

Earlier quoted context omitted.

No, that was 20-30 years ago.

At this point, the best prevention is to avoid eating anyone who lived in Britain 20-30 years ago

You forgot anyone who ate anyone who lived in Britain 20-30 years ago.