Axonal prion protein is required for peripheral myelin maintenance

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