Question about chaperonins and disease

[banaji727]

What do chaperonins have to do with health like mad cow disease proteins called prions?

I seriously have no idea. I know chaperonins allow polypeptides to fold without outside disturbances but that's as far as I can get.

[biohazard]

I'm not certain about this, but I think it could be that mutated/malfunctioning chaperonens may in certain cases misfold proteins and turn them into prions. Prions, then, seem to have some kind of chaperon-like properties that allow them to misfold other proteins of their kind, causing plaques typical to dieases like the mad cow's. Alternatively, all the prionic proteins might be created by dysfunctional chaperons.

[kolean]

Do they really fold without outside disturbances? I would consider the chaperonins to be an outside disturbance.

I thought chaperonins help the protein to fold in a way that is condusive to its ultimate function. That yes, it does keep out other influences, say water molecules and other polar substances that could influence the folding of the peptide, as it helps it fold into a fully functional protein. So if the chaperonins are mutated, then the folding of the protein would be hindered if it was in a site that was condusive to the folding pattern. Then disease would be the outcome of that mutation.

[MrMistery]

a mutated chaperonin would indeed result in increased protein misfolding. However, I don't think it would generate the Pr^sc version of the prion protein. As far as I know, the only thing that can cause the prion protein (which is present in every individual's brain) to fold in its toxic state is another prion protein. That's why people don't just get prion diseases, you need to get them from coming into contact with the infectious prion protein.

[jonmoulton]

Basic rule for neuroprotein health - don't eat human brains, don't pick your nose when preparing human brains for other's consumption, don't wallow in human brains - in fact, leave human brains in their skulls (unless you are a neurosurgeon and must expose yourself to human brain tissue, a necessary job hazard, and they wear gloves and masks; watch that spatter from the drill!).
http://en.wikipedia.org/wiki/Kuru_%28disease%29

[JackBean]

Basic rule for neuroprotein health - don't eat human brains, don't pick your nose when preparing human brains for other's consumption, don't wallow in human brains - in fact, leave human brains in their skulls (unless you are a neurosurgeon and must expose yourself to human brain tissue, a necessary job hazard, and they wear gloves and masks; watch that spatter from the drill!).
http://en.wikipedia.org/wiki/Kuru_%28disease%29

Thats definitely not only about human brains. In UK there was a case, when some buther used on knife for killing whole cows, so people not even eating their brains, did get some of the prions from meat.