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Prions Increasingly Implicated in Neurodegenerative Diseases

Discussion in 'Other Health News and Research' started by leokitten, Sep 1, 2015.

  1. leokitten

    leokitten Senior Member

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    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
     
    Last edited: Sep 1, 2015
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  2. A.B.

    A.B. Senior Member

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    Dr de Meirleir also thinks that aberrant prions play a role in ME/CFS, especially in the severe cases.
     
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  3. rosie26

    rosie26 Senior Member

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    It passed through my mind in my severe years. The feeling in the body and legs. Especially, the legs.
     
    Last edited: Sep 1, 2015
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  4. lansbergen

    lansbergen Senior Member

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

    lansbergen Senior Member

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  6. Martial

    Martial Senior Member

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

    lansbergen Senior Member

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    Yes,THE protein.
     
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  8. alkt

    alkt Senior Member

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    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 .
     
  9. lansbergen

    lansbergen Senior Member

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    With all the measures taken food is less risky for TSE infection then medical treatments.
     
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  10. alex3619

    alex3619 Senior Member

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    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.
     
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  11. lansbergen

    lansbergen Senior Member

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    TSE prions are found in almost every tissue.
     
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  12. alex3619

    alex3619 Senior Member

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    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.
     
  13. lansbergen

    lansbergen Senior Member

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    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.
     

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