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The sympathetic nervous system is involved in variant Creutzfeldt-Jakob disease

Nature Medicine volume 9, pages 1121–1122 (2003)Cite this article

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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Authors and Affiliations

  1. Raymond Escourolle Neuropathology Laboratory, Association Claude Bernard and INSERM U360, Pitié-Salpêtrière Hospital, Paris, 75013, France

    Stéphane Haïk, Baptiste A Faucheux, Véronique Sazdovitch, Nicolas Privat & Jean-Jacques Hauw

  2. Department of Anatomy and Pathology, G. Montpied Hospital, Clermond-Ferrand, 63000, France

    Jean-Louis Kemeny

  3. Center for Diagnosis and Treatment of Prions Diseases, Biochemistry Laboratory, Neurological Hospital, Lyon, 69003, France

    Armand Perret-Liaudet

Authors
  1. Stéphane Haïk
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  2. Baptiste A Faucheux
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  3. Véronique Sazdovitch
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  4. Nicolas Privat
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  6. Armand Perret-Liaudet
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  7. Jean-Jacques Hauw
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Correspondence to Stéphane Haïk.

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The authors declare no competing financial interests.

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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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