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Cellular prion protein is expressed in the human enteric nervous system

Nature Medicine volume 6, pages 840–841 (2000)Cite this article

To the editor

Oral exposure to the bovine spongiform encephalopathy agent is thought to be responsible for the recent emergence of variant Creutzfeldt-Jakob disease in the UK population1. It is also a possible natural transmission route for other transmissible spongiform encephalopathies (TSEs). A host prion protein, PrPc, is essential for TSE pathogenesis and is abundantly expressed in the central nervous system, where it may be conformationally modified during disease into an aberrant protease-resistant form (PrPSc)2. In an orally challenged rodent TSE model, PrPSc is detected early in the enteric nervous system (ENS)3. However, there are no published studies on PrPc expression in the ENS, either in humans or animals. Mapping the precise anatomical and cellular distribution of PrPc is essential to any complete analysis of its function.

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Figure 1: Expression of cellular prion protein and its mRNA in neurons and glial cells of the ENS.

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Authors and Affiliations

  1. Department of Medical Sciences, Gastrointestinal Laboratory, University of Edinburgh, Western General Hospital, Edinburgh, EH4 2XU, Scotland, UK

    Andrei N. Shmakov, John Bode & Subrata Ghosh

  2. National CJD Surveillance Unit, University of Edinburgh, Western General Hospital, Edinburgh, EH4 2XU, Scotland, UK

    Neil F. McLennan & Kathleen A. Rennison

  3. Neuropathogenesis Unit, Institute for Animal Health, Ogston Building, Edinburgh, EH9 3JF, Scotland, UK

    Patricia McBride & Christine F. Farquhar

Authors
  1. Andrei N. Shmakov
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  2. Neil F. McLennan
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  3. Patricia McBride
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  4. Christine F. Farquhar
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  5. John Bode
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  6. Kathleen A. Rennison
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  7. Subrata Ghosh
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Shmakov, A., McLennan, N., McBride, P. et al. Cellular prion protein is expressed in the human enteric nervous system. Nat Med 6, 840–841 (2000). https://doi.org/10.1038/78558

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