Chronic wasting disease prion trafficking via the autonomic nervous system

Davis M. Seelig, Gary L. Mason, Glenn C. Telling, Edward A. Hoover

Research output: Contribution to journalArticlepeer-review

21 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)1319-1328
Number of pages10
JournalAmerican Journal of Pathology
Issue number3
StatePublished - Sep 2011
Externally publishedYes

Bibliographical note

Funding Information:
Supported by NIH contract NIAID-N01-AI-25491 (E.A.H.) and training grant NCRR-T32-RR07072 (D.M.S.).


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