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Accumulation of prion protein in the vagus nerve in creutzfeldt–jakob disease

Disease‐associated proteins are thought to propagate along neuronal processes in neurodegenerative diseases. To detect disease‐associated prion protein (PrPSc) in the vagus nerve in different forms and molecular subtypes of Creutzfeldt–Jakob disease (CJD), we applied 3 different anti‐PrP antibodies....

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Published in:Annals of neurology 2019-05, Vol.85 (5), p.782-787
Main Authors: Kresl, Philip, Rahimi, Jasmin, Gelpi, Ellen, Aldecoa, Iban, Ricken, Gerda, Danics, Krisztina, Keller, Eva, Kovacs, Gabor G.
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description Disease‐associated proteins are thought to propagate along neuronal processes in neurodegenerative diseases. To detect disease‐associated prion protein (PrPSc) in the vagus nerve in different forms and molecular subtypes of Creutzfeldt–Jakob disease (CJD), we applied 3 different anti‐PrP antibodies. We screened the vagus nerve in 162 sporadic and 30 genetic CJD cases. Four of 31 VV‐2 type sporadic CJD and 7 of 30 genetic CJD cases showed vagal PrPSc immunodeposits with distinct morphology. Thus, PrPSc in CJD affects the vagus nerve analogously to α‐synuclein in Parkinson disease. The morphologically diverse deposition of PrPSc in genetic and sporadic CJD argues against uniform mechanisms of propagation of PrPSc. Ann Neurol 2019;85:782–787
doi_str_mv 10.1002/ana.25451
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subjects Antibodies
Bovine spongiform encephalopathy
Brief Communication
Brief Communications
Cohort Studies
Creutzfeldt-Jakob disease
Creutzfeldt-Jakob Syndrome - metabolism
Creutzfeldt-Jakob Syndrome - pathology
Disease
Humans
Morphology
Movement disorders
Neurodegenerative diseases
Neurological diseases
Parkinson's disease
Prion protein
Prion Proteins - analysis
Prion Proteins - metabolism
Proteins
Retrospective Studies
Synuclein
Vagus nerve
Vagus Nerve - chemistry
Vagus Nerve - metabolism
Vagus Nerve - pathology
title Accumulation of prion protein in the vagus nerve in creutzfeldt–jakob disease
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