ARS | Publication request: Myenteric neurons of the ileum that express somatostatin are a target of prion neuroinvasion in an alimentary model of sheep scrapie

Submitted to: Acta Neuropathologica
Publication Type: Peer Reviewed Journal
Publication Acceptance Date: March 22, 2008
Publication Date: June 2, 2008
Citation: Schneider, D.A., Yan, H., Fry, L.M., Alverson, J., White, S.N., Orourke, K.I. 2008. Myenteric neurons of the ileum that express somatostatin are a target of prion neuroinvasion in an alimentary model of sheep scrapie. Acta Neuropathologica. 115(6):651-661.

Interpretive Summary: This manuscript evaluates the process of prion invasion of a peripheral nervous system in a model of natural disease transmission in sheep. The data show that peripheral neuroinvasion is a process associated with risk factors inherent to certain types of neuron early in disease progression and before detection of dissemination to the central nervous system.

Technical Abstract: Scrapie disease is an orally transmissible spongiform encephalopathy disease of sheep in which the causative agent, the prion, can disseminate to the brain via nerves innervating the gut. Nerve cells intrinsic to the ileum (enteric neurons) are the first part of the peripheral nervous system invaded by scrapie prions, detected as the immunoreactive presence of PrP-Sc. Following oral inoculation of highly-susceptible lambs with infectious placenta, prion invasion of enteric neurons in the ileum was first detectable in enteric neurons at about 7 months of age. Certain enteric neural subpopulations were found to be either at increased risk or at decreased risk of invasion by scrapie prions and were partially characterized by immunohistochemistry.