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Journal of Virology, July 2004, p. 6792-6798, Vol. 78, No. 13
0022-538X/04/$08.00+0 DOI: 10.1128/JVI.78.13.6792-6798.2004
Copyright © 2004, American Society for Microbiology. All Rights Reserved.
Prion Infection of Skeletal Muscle Cells and Papillae in the Tongue
Ellyn R. Mulcahy,1,
Jason C. Bartz,1 Anthony E. Kincaid,2 and Richard A. Bessen3*
Department of Medical Microbiology and Immunology,1
Department of Physical Therapy, Creighton University, Omaha, Nebraska 68178,2
Department of Veterinary Molecular Biology, Montana State University Bozeman, Montana 597173
Received 29 September 2003/
Accepted 3 March 2004
The presence of the prion agent in skeletal muscle is thought to be due to the infection of nerve fibers located within the muscle. We report here that the pathological isoform of the prion protein, PrPSc, accumulates within skeletal muscle cells, in addition to axons, in the tongue of hamsters following intralingual and intracerebral inoculation of the HY strain of the transmissible mink encephalopathy agent. Localization of PrPSc to the neuromuscular junction suggests that this synapse is a site for prion agent spread between motor axon terminals and muscle cells. Following intracerebral inoculation, the majority of PrPSc in the tongue was found in the lamina propria, where it was associated with sensory nerve fibers in the core of the lingual papillae. PrPSc staining was also identified in the stratified squamous epithelium of the lingual mucosa. These findings indicate that prion infection of skeletal muscle cells and the epithelial layer in the tongue can be established following the spread of the prion agent from nerve terminals and/or axons that innervate the tongue. Our data suggest that ingestion of meat products containing prion-infected tongue could result in human exposure to the prion agent, while sloughing of prion-infected epithelial cells at the mucosal surface of the tongue could be a mechanism for prion agent shedding and subsequent prion transmission in animals.
* Corresponding author. Mailing address: Department of Veterinary Molecular Biology, P.O. Box 173610, Montana State University, Bozeman, MT 59717. Phone: (406) 994-1563. Fax: (406) 994-4303. E-mail:
rbessen{at}montana.edu.
Present address: Department of Anatomy and Cell Biology, University of Kansas Medical Center, Kansas City, KS 66160.
Journal of Virology, July 2004, p. 6792-6798, Vol. 78, No. 13
0022-538X/04/$08.00+0 DOI: 10.1128/JVI.78.13.6792-6798.2004
Copyright © 2004, American Society for Microbiology. All Rights Reserved.
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