title: 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.
authors.affiliation: Raymond Escourolle Neuropathology Laboratory, Association Claude Bernard and INSERM U360, Pitié-Salpêtrière Hospital, 75013, Paris, France. haik@mailhost.chups.jussieu.fr
authors.forename: Stéphane
authors.initials: S
authors.lastname: Haïk
authors.forename: Baptiste A
authors.initials: BA
authors.lastname: Faucheux
authors.forename: Véronique
authors.initials: V
authors.lastname: Sazdovitch
authors.forename: Nicolas
authors.initials: N
authors.lastname: Privat
authors.forename: Jean-Louis
authors.initials: JL
authors.lastname: Kemeny
authors.forename: Armand
authors.initials: A
authors.lastname: Perret-Liaudet
authors.forename: Jean-Jacques
authors.initials: JJ
authors.lastname: Hauw
mesh_terms: D000328:Adult; D002395:Catecholamines / Q000378:metabolism; D007562:Creutzfeldt-Jakob Syndrome / Q000209:etiology* / Q000503:physiopathology*; D005260:Female; D005728:Ganglia, Sympathetic / Q000378:metabolism; D006801:Humans; D008279:Magnetic Resonance Imaging; D009474:Neurons / Q000378:metabolism* / Q000473:pathology; D018620:PrPSc Proteins / Q000378:metabolism*; D013233:Stellate Ganglion / Q000378:metabolism; D013564:Sympathetic Nervous System / Q000378:metabolism* / Q000473:pathology
chemical_list: D002395:Catecholamines; D018620:PrPSc Proteins