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Publication : Chronic wasting disease prion trafficking via the autonomic nervous system.

First Author  Seelig DM Year  2011
Journal  Am J Pathol Volume  179
Issue  3 Pages  1319-28
PubMed ID  21777560 Mgi Jnum  J:176313
Mgi Id  MGI:5290032 Doi  10.1016/j.ajpath.2011.05.057
Citation  Seelig DM, et al. (2011) Chronic wasting disease prion trafficking via the autonomic nervous system. Am J Pathol 179(3):1319-28
abstractText  Chronic wasting disease (CWD) is a fatal spongiform encephalopathy that is efficiently transmitted among members of the mammalian family Cervidae, including deer, elk, and moose. Typical of prion diseases, CWD is characterized by the conversion of the native protease-sensitive protein PrP(C) to a protease-resistant isoform, denoted PrP(RES). In native species, spread of the disease likely results from the ingestion of prion-containing excreta, including urine, saliva, or feces. Although cervid prion protein-expressing transgenic [Tg(CerPrP)] mice have been shown to be effective surrogates of natural CWD, uncertainties remain regarding the mechanisms by which CWD prions traffic in vivo, including the manner by which CWD prions traffic from the gastrointestinal tract to the central nervous system. We used elk prion protein-expressing transgenic [Tg(CerPrP-E)] mice, infected by three different routes of inoculation, and tissue-based IHC to elucidate that centripetal and centrifugal CWD prion transit pathways involve cells and fibers of the autonomic nervous systems, including the enteric nervous system and central autonomic network. Moreover, we identified CWD PrP(RES) associated with the cell bodies and processes of enteric glial cells within the enteric nervous system of CWD-infected Tg(CerPrP-E) mice. The present findings demonstrate the importance of the peripheral and central autonomic networks in CWD neuroinvasion and neuropathogenesis and suggest that enteroglial cells may facilitate the shedding of prions via the intestinal tract.
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