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PrPC - Prion ESSENTIAL for myelin maintenance

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    PrPC - Prion ESSENTIAL for myelin maintenance

    There is a big push by members of the agri-scientife community, including our own Alberta Prion Institute, to push for genetically modified cattle, ie: cloned cattle, that are "prion-free". Meaning, that they would alter the genetic material of a bovine embryo, to shut off production of the prion protein.

    Well, this won't work!! First, the prion is necessary for cell differentiation in the embryonic stages, and any subsequent stem cell maturation, [this is why they have to shut the gene down after differentiation].... and now, here is more evidence about how vital the prion is in nerve health.

    The myelin sheath surrounding the nerve will undergo chronic demyelinating polyneuropathy (CDP), when this "essential protein" (PrPC) is shut-off.


    "Nat Neurosci. 2010 Jan 24. [Epub ahead of print]

    Axonal prion protein is required for peripheral myelin maintenance.
    Bremer J, Baumann F, Tiberi C, Wessig C, Fischer H, Schwarz P, Steele AD, Toyka KV, Nave KA, Weis J, Aguzzi A.

    Institute of Neuropathology, University Hospital of Zürich, Zürich, Switzerland.

    The integrity of peripheral nerves relies on communication between axons and Schwann cells. The axonal signals that ensure myelin maintenance are distinct from those that direct myelination and are largely unknown. Here we show that ablation of the prion protein PrP(C) triggers a chronic demyelinating polyneuropathy (CDP) in four independently targeted mouse strains. Ablation of the neighboring Prnd locus, or inbreeding to four distinct mouse strains, did not modulate the CDP. CDP was triggered by depletion of PrP(C) specifically in neurons, but not in Schwann cells, and was suppressed by PrP(C) expression restricted to neurons but not to Schwann cells. CDP was prevented by PrP(C) variants that undergo proteolytic amino-proximal cleavage, but not by variants that are nonpermissive for cleavage, including secreted PrP(C) lacking its glycolipid membrane anchor. These results indicate that neuronal expression and regulated proteolysis of PrP(C) are essential for myelin maintenance.

    PMID: 20098419"

    Demyelination of the nerve is a marker for Multiple Sclerosis. It is interesting to see the research out of Italy where-in, a researcher demonstrated that by unclogging blocked veins in the neck, MS patients were able to regain myelin integrity and improve their health. The blocking of neck vein, in some patient's, restricted the availability of oxygen and nutrients to the adjacent cells.
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