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Link to original content: https://doi.org/10.1038/nm922
The sympathetic nervous system is involved in variant Creutzfeldt-Jakob disease | Nature Medicine
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The sympathetic nervous system is involved in variant Creutzfeldt-Jakob disease

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.

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Figure 1: PrPsc accumulation in sympathetic ganglia of vCJD, as shown by immunohistochemistry.
Figure 2: PET-blot and western blot in sympathetic ganglia of vCJD.

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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).

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Correspondence to Stéphane Haïk.

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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

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