The sympathetic nervous system is involved in variant Creutzfeldt-Jakob disease.
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.
Haïk
Stéphane
S
Raymond Escourolle Neuropathology Laboratory, Association Claude Bernard and INSERM U360, Pitié-Salpêtrière Hospital, 75013, Paris, France. haik@mailhost.chups.jussieu.fr
Faucheux
Baptiste A
BA
Sazdovitch
Véronique
V
Privat
Nicolas
N
Kemeny
Jean-Louis
JL
Perret-Liaudet
Armand
A
Hauw
Jean-Jacques
JJ
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
D002395:Catecholamines; D018620:PrPSc Proteins