Postmortem Quantitative Analysis of Prion Seeding Activity in the Digestive System

Human prion diseases are neurodegenerative disorders caused by prion protein. Although infectivity was historically detected only in the central nervous system and lymphoreticular tissues of patients with sporadic Creutzfeldt-Jakob disease, recent reports suggest that the seeding activity of Creutzf...

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Bibliographic Details
Published in:Molecules (Basel, Switzerland) Switzerland), 2019-12, Vol.24 (24), p.4601
Main Authors: Satoh, Katsuya, Fuse, Takayuki, Nonaka, Toshiaki, Dong, Trong, Takao, Masaki, Nakagaki, Takehiro, Ishibashi, Daisuke, Taguchi, Yuzuru, Mihara, Ban, Iwasaki, Yasushi, Yoshida, Mari, Nishida, Noriyuki
Format: Article
Language:English
Subjects:
Adult
Aged
Animal models
Animals
Brain research
Case-Control Studies
Creutzfeldt-Jakob disease
Creutzfeldt-Jakob Syndrome - metabolism
Creutzfeldt-Jakob Syndrome - pathology
Digestive system
Digestive System - metabolism
Digestive System - pathology
Encephalitis
Epithelium
Esophagus
Female
Humans
Immunohistochemistry
Infections
Infectivity
Male
Middle Aged
Olfactory epithelium
Organ Specificity
Ostomy
Patients
Postmortem Changes
Prion Diseases - metabolism
Prion Diseases - pathology
Prion protein
Prions
Prions - metabolism
Protein seeding
Proteins
Quantitative analysis
Reabsorption
Scrapie
Spleen
Stomach
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Description
Summary:Human prion diseases are neurodegenerative disorders caused by prion protein. Although infectivity was historically detected only in the central nervous system and lymphoreticular tissues of patients with sporadic Creutzfeldt-Jakob disease, recent reports suggest that the seeding activity of Creutzfeldt-Jakob disease prions accumulates in various non-neuronal organs including the liver, kidney, and skin. Therefore, we reanalyzed autopsy samples collected from patients with sporadic and genetic human prion diseases and found that seeding activity exists in almost all digestive organs. Unexpectedly, activity in the esophagus reached a level of prion seeding activity close to that in the central nervous system in some CJD patients, indicating that the safety of endoscopic examinations should be reconsidered.
ISSN:1420-3049
1420-3049
DOI:10.3390/molecules24244601