Interesting, and somewhat horrifying, prion-caused disease.

http://www.bbc.com/future/story/20160118-the-tragic-fate-of-the-people-who-stop-sleeping

https://en.m.wikipedia.org/wiki/Fatal_familial_insomnia

dv said:

Interesting, and somewhat horrifying, prion-caused disease.

http://www.bbc.com/future/story/20160118-the-tragic-fate-of-the-people-who-stop-sleeping

https://en.m.wikipedia.org/wiki/Fatal_familial_insomnia

Nightmarish.

I was not previously familiar with familially transmitted prions. Given that it is not in the nucleic DNA, I wonder what the mechanism is.

dv said:

I was not previously familiar with familially transmitted prions. Given that it is not in the nucleic DNA, I wonder what the mechanism is.

The prion itself isn’t inherited; the mutated gene that codes for a mal-folded protein is inherited.

OCDC said:

dv said:

I was not previously familiar with familially transmitted prions. Given that it is not in the nucleic DNA, I wonder what the mechanism is.

The prion itself isn’t inherited; the mutated gene that codes for a mal-folded protein is inherited.

And this malfolded protein IS the prion?

dv said:

OCDC said:

dv said:

I was not previously familiar with familially transmitted prions. Given that it is not in the nucleic DNA, I wonder what the mechanism is.

The prion itself isn’t inherited; the mutated gene that codes for a mal-folded protein is inherited.

And this malfolded protein IS the prion?

Yes. Same protein but different fold causes CJD.

The mechanism in vCJD (the one you get from mad cows) and kuru is thought to be one malfolded protein touching a properly folded protein thereby making it change its tertiary structure.

The Prion Alliance website explains it well.

OCDC said:

dv said:

OCDC said:

The prion itself isn’t inherited; the mutated gene that codes for a mal-folded protein is inherited.

And this malfolded protein IS the prion?

Yes. Same protein but different fold causes CJD.

The mechanism in vCJD (the one you get from mad cows) and kuru is thought to be one malfolded protein touching a properly folded protein thereby making it change its tertiary structure.

Cheers.

There is a whole world of no out there.

OCDC said:

dv said:

OCDC said:

The prion itself isn’t inherited; the mutated gene that codes for a mal-folded protein is inherited.

And this malfolded protein IS the prion?

Yes. Same protein but different fold causes CJD.

The mechanism in vCJD (the one you get from mad cows) and kuru is thought to be one malfolded protein touching a properly folded protein thereby making it change its tertiary structure.

So how does eating affected meat cause CJD? Blood transfer?

Arts said:

OCDC said:

dv said:

And this malfolded protein IS the prion?

Yes. Same protein but different fold causes CJD.

The mechanism in vCJD (the one you get from mad cows) and kuru is thought to be one malfolded protein touching a properly folded protein thereby making it change its tertiary structure.

So how does eating affected meat cause CJD? Blood transfer?

It’s unclear. The prion shouldn’t be able to get through the blood-brain barrier which manages to block many much smaller molecules.

OCDC said:

Arts said:

OCDC said:

Yes. Same protein but different fold causes CJD.

The mechanism in vCJD (the one you get from mad cows) and kuru is thought to be one malfolded protein touching a properly folded protein thereby making it change its tertiary structure.

So how does eating affected meat cause CJD? Blood transfer?

It’s unclear. The prion shouldn’t be able to get through the blood-brain barrier which manages to block many much smaller molecules.

predisposition? Some genetic malfunction not a problem until introduction… Curious.

OCDC said:

The Prion Alliance website explains it well.

Yes, yes it does. Thanks for the link.

I’m still annoyed that no-one has yet marketed a cure for prion diseases.

mollwollfumble said:

I’m still annoyed that no-one has yet marketed a cure for prion diseases.

When did you become annoyed about that?

mollwollfumble said:

I’m still annoyed that no-one has yet marketed a cure for prion diseases.

Big pharma doesn’t want cures only “medicine” that prolonges life until you have no more money.

Cymek said:

mollwollfumble said:

I’m still annoyed that no-one has yet marketed a cure for prion diseases.

Big pharma doesn’t want cures only “medicine” that prolonges life until you have no more money.

Big pharma tries to not waste money on small scale problems like these…

Bubblecar said:

dv said:

Interesting, and somewhat horrifying, prion-caused disease.

http://www.bbc.com/future/story/20160118-the-tragic-fate-of-the-people-who-stop-sleeping

https://en.m.wikipedia.org/wiki/Fatal_familial_insomnia

Nightmarish.

Unlikely.

zing

Michael V said:

mollwollfumble said:

I’m still annoyed that no-one has yet marketed a cure for prion diseases.

When did you become annoyed about that?

I can answer that, I first became annoyed about the slowness of developing a cure for prion diseases in the year 1989.

mollwollfumble said:

Michael V said:

mollwollfumble said:

I’m still annoyed that no-one has yet marketed a cure for prion diseases.

When did you become annoyed about that?

I can answer that, I first became annoyed about the slowness of developing a cure for prion diseases in the year 1989.

Wow! I probably wasn’t even thinking about it except Mrs V was not permitted to give blood for many years. And that annoyed her.