Prions Increasingly Implicated in Neurodegenerative Diseases

[leokitten]

Big science news this week, Stanley Prusiner's lab at UCSF (he won Nobel prize 1997 for discovery of prions) just published two big papers.

First the discovery of α-synuclein prions and that they cause multiple system atrophy (MSA):

Evidence for α-synuclein prions causing multiple system atrophy in humans with parkinsonism

Second just like BSE ("mad cow") and CJD it seems that MSA is potentially transmissible:

Propagation of prions causing synucleinopathies in cultured cells

His lab is finding evidence that different prions cause neurodegenerative diseases such as progressive supranuclear palsy (PSP) and even Parkinson's disease (PD).

Additional info here:
New Type of Prion May Cause, Transmit Neurodegeneration

 

[A.B.]

Dr de Meirleir also thinks that aberrant prions play a role in ME/CFS, especially in the severe cases.

 

[rosie26]

It passed through my mind in my severe years. The feeling in the body and legs. Especially, the legs.

 

[lansbergen]

A misfolded protein is not a prion if it is not infectious.

 

[lansbergen]

https://en.wikipedia.org/wiki/Prion

The word prion, coined in 1982 by Stanley B. Prusiner, is derived from the words protein and infection,

 

[Martial]

isn't prions one of the proteins that is a part of mad cow disease?

 

[lansbergen]

isn't prions one of the proteins that is a part of mad cow disease?

Yes,THE protein.

 

[alkt]

is there not enough medically scary things going on without giving people with severe cognative disfunction nightmares. have to say it is interesting though .what other prions might turn up in our food chain .thanks to the food industries never ceasing demand for greater profits .

 

[lansbergen]

is there not enough medically scary things going on without giving people with severe cognative disfunction nightmares. have to say it is interesting though .what other prions might turn up in our food chain .thanks to the food industries never ceasing demand for greater profits .

With all the measures taken food is less risky for TSE infection then medical treatments.

 

[alex3619]

Misfolded proteins are normal. They occur all the time. We have mechanisms to remove them. What makes prions dangerous is they interact with differently folded versions of the same protein and refold them. In other words, its like cloning. They make more of themselves. If that prion protein is difficult to degrade for some reason then it can accumulate. So you get an increase in useless protein in that tissue type where it is expressed, and a deficiency of the properly working protein. An analogy is cholesterol and lesions clogging an artery and stopping blood to the heart, but a prion stops the working machinery of the cell from doing its job. The cell can stop working, and even die, releasing the prions to the surrounding tissue. The cell might also be killed by the immune response, but this might just release the prions.

I expect to see oodles of misfolded proteins in ME. This can occur simply by high oxidative stress. Reduced glutathione is necessary for proper protein folding. Whether they replicate is an entirely different story.

By implication, though we have found a prion responsible for at least two neurodegenerative disorders (which might really be one disorder but labelled badly) , its likely there are prions that affect other tissue types that await discovery.

 

[lansbergen]

its likely there are prions that affect other tissue types that await discovery.

TSE prions are found in almost every tissue.

 

[alex3619]

My understanding is that the protein distribution is ubiquitous, but that the prion is only found in detectable levels in the brain. This might be because the levels are just too low elsewhere, or I have not read enough, or that the brain lacks sufficient capacity to remove these prions.

 

[lansbergen]

My understanding is that the protein distribution is ubiquitous, but that the prion is only found in detectable levels in the brain. This might be because the levels are just too low elsewhere, or I have not read enough, or that the brain lacks sufficient capacity to remove these prions.

I think you are running behind. Science evolves. Now they can even be found in nose mucosa, eye lids and the rectum.

Tests are not yet sensitive enough to find cariiers that way but they are working hard on it.