Abstract
Prion epizoonoses spread from animals consumed by humans raise the question of which pathways lead to prion neuroinvasion after oral exposure of humans. Here we show that neurons of sympathetic ganglia of patients with variant Creutzfeldt-Jakob disease (vCJD) accumulate the abnormal isoform of the protein prion. This observation shows the involvement of the sympathetic nervous system in the pathogenesis of vCJD and suggests a role for GUT-associated sympathetic neurons in prion propagation in humans after oral contamination.
This is a preview of subscription content, access via your institution
Access options
Subscribe to this journal
Receive 12 print issues and online access
$209.00 per year
only $17.42 per issue
Buy this article
- Purchase on SpringerLink
- Instant access to full article PDF
Prices may be subject to local taxes which are calculated during checkout
Similar content being viewed by others
References
Will, R.G. et al. Lancet 347, 921–925 (1996).
Bruce, M.E. et al. Nature 389, 498–501 (1997).
Aguzzi, A., Montrasio, F. & Kaeser, P.S. Nat. Rev. Mol. Cell. Biol. 2, 118–126 (2001).
Glatzel, M., Heppner, F.L., Albers, K.M. & Aguzzi, A. Neuron 31, 25–34 (2001).
McBride, P.A. et al. J. Virol. 75, 9320–9327 (2001).
Shmakov, A.N. et al. Nat. Med. 6, 840–841 (2000).
Oppenheim, C., Brandel, J., Hauw, J.J., Deslys, J.P. & Fontaine, B. Lancet 356, 253–254 (2000).
West, M.J. Neurobiol. Aging 14, 287–293 (1993).
Goto, S. & Hirano, A. Ann. Neurol. 30, 192–196 (1991).
Schulz-Schaeffer, W.J. et al. Am. J. Pathol. 156, 51–56 (2000).
Koperek, O. et al. Am. J. Pathol. 161, 1979–1984 (2002).
Collinge, J., Sidle, K.C.L., Meads, J., Ironside, J. & Hill, A.F. Nature 383, 685–690 (1996).
Parchi, P. et al. Ann. Neurol. 39, 767–778 (1996).
Kovacs, G.G. et al. Brain Pathol. 12, 1–11 (2002).
Haïk, S., Dormont, D., Faucheux, B.A., Marsault, C. & Hauw, J.J. Ann. Neurol. 51, 797–799 (2002).
Acknowledgements
We thank D.L. Ritchie and J.W. Ironside (National CJD Surveillance Unit, Edinburgh University, UK) for kindly providing the PET-blot procedure, and F. Mallie-Didier and A.G. Biacabe (Biochemistry Laboratory, Neurological Hospital, Lyon, France) for their technical help. This study was supported in part by the European Union Concerted Action on Human Transmissible Spongiform Encephalopathies (Biomed 2, contract BMH4-CT98-6003), the Groupement d'Intérêt Scientifique—Infections à Prions and INSERM (ATC Prions).
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Competing interests
The authors declare no competing financial interests.
Rights and permissions
About this article
Cite this article
Haïk, S., Faucheux, B., Sazdovitch, V. et al. The sympathetic nervous system is involved in variant Creutzfeldt-Jakob disease. Nat Med 9, 1121–1122 (2003). https://doi.org/10.1038/nm922
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1038/nm922
This article is cited by
-
Prion type 2 selection in sporadic Creutzfeldt–Jakob disease affecting peripheral ganglia
Acta Neuropathologica Communications (2021)
-
The immunobiology of prion diseases
Nature Reviews Immunology (2013)
-
Severe and rapidly evolving peripheral neuropathy revealing sporadic Creutzfeldt-Jakob disease
Journal of Neurology (2009)
-
Anatomical evidence for ileal Peyer’s patches innervation by enteric nervous system: a potential route for prion neuroinvasion?
Cell and Tissue Research (2008)
-
Application of Atomic Dielectric Resonance Spectroscopy for the screening of blood samples from patients with clinical variant and sporadic CJD
Journal of Translational Medicine (2007)