1. Patients launch $1.27 million crowdfunding campaign for ME/CFS gut microbiome study.
    Check out the website, Facebook and Twitter. Join in donate and spread the word!
August 8th - What is the one thing about suffering with severe ME that the world needs to know?
Andrew Gladman brings our coverage of the Understanding & Remembrance Day for Severe ME, airing the voice of patients ...
Discuss the article on the Forums.

peripher polyneuropathy and PrPc

Discussion in 'Other Health News and Research' started by lansbergen, Jan 4, 2014.

  1. lansbergen

    lansbergen Senior Member

    Messages:
    1,035
    Likes:
    498
    http://www.nature.com/neuro/journal/v13/n3/full/nn.2483.html

    Axonal prion protein is required for peripheral myelin maintenance

    Abstract

    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 PrPC 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 PrPC specifically in neurons, but not in Schwann cells, and was suppressed by PrPC expression restricted to neurons but not to Schwann cells. CDP was prevented by PrPC variants that undergo proteolytic amino-proximal cleavage, but not by variants that are nonpermissive for cleavage, including secreted PrPC lacking its glycolipid membrane anchor. These results indicate that neuronal expression and regulated proteolysis of PrPC are essential for myelin maintenance.

See more popular forum discussions.

Share This Page