August 29, 2004 Medical Letter on the CDC & FDA

According to a study from the United States, "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, PrP, 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 PrP 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 PrP in the tongue was found in the lamina propria, where it was associated with sensory nerve fibers in the core of the lingual papillae," said Ellyn R. Mulcahy and collaborators at Creighton University and Montana State University. "PrP staining was also identified in the stratified squamous epithelium of the lingual mucosa."

The researchers concluded, "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."

Mulcahy and associates published their study in the Journal of Virology (Prion infection of skeletal muscle cells and papillae in the tongue. J Virol, 2004;78(13):6792-6798).

For more information, contact Richard A. Bessen, Department of Veterinary Molecular Biology, Montana State University, PO Box 173610, Bozeman, MT 59717, USA. E-mail: rbessen@montana.edu.

Publisher contact information for the Journal of Virology is: American Society for Microbiology, 1752 N Street NW, Washington, DC 20036-2904, USA.