Prion-Seeding Activity Is widely Distributed in Tissues of Sporadic Creutzfeldt-Jakob Disease Patients

Human prion diseases are neurodegenerative disorders caused by abnormally folded prion proteins in the central nervous system. These proteins can be detected using the quaking-induced conversion assay. Compared with other bioassays, this assay is extremely sensitive and was used in the present study...

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Main Authors: Hanae Takatsuki, PhD, Takayuki Fuse, PhD, Takehiro Nakagaki, MD, PhD, Tsuyoshi Mori, PhD, Ban Mihara, MD, PhD, Masaki Takao, MD, PhD, Yasushi Iwasaki, MD, PhD, Mari Yoshida, MD, PhD, Shigeo Murayama, MD, PhD, Ryuichiro Atarashi, MD, PhD, Noriyuki Nishida, MD, PhD, Katsuya Satoh, MD, PhD
Format: Article
Language:English
Published: Elsevier 2016-10-01
Series:EBioMedicine
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S2352396416303887
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author Hanae Takatsuki, PhD
Takayuki Fuse, PhD
Takehiro Nakagaki, MD, PhD
Tsuyoshi Mori, PhD
Ban Mihara, MD, PhD
Masaki Takao, MD, PhD
Yasushi Iwasaki, MD, PhD
Mari Yoshida, MD, PhD
Shigeo Murayama, MD, PhD
Ryuichiro Atarashi, MD, PhD
Noriyuki Nishida, MD, PhD
Katsuya Satoh, MD, PhD
author_facet Hanae Takatsuki, PhD
Takayuki Fuse, PhD
Takehiro Nakagaki, MD, PhD
Tsuyoshi Mori, PhD
Ban Mihara, MD, PhD
Masaki Takao, MD, PhD
Yasushi Iwasaki, MD, PhD
Mari Yoshida, MD, PhD
Shigeo Murayama, MD, PhD
Ryuichiro Atarashi, MD, PhD
Noriyuki Nishida, MD, PhD
Katsuya Satoh, MD, PhD
author_sort Hanae Takatsuki, PhD
collection DOAJ
description Human prion diseases are neurodegenerative disorders caused by abnormally folded prion proteins in the central nervous system. These proteins can be detected using the quaking-induced conversion assay. Compared with other bioassays, this assay is extremely sensitive and was used in the present study to determine prion distribution in sporadic Creutzfeldt-Jakob disease patients at autopsy. Although infectivity of the sporadic form is thought to be restricted within the central nervous system, results showed that prion-seeding activities reach 106/g from a 50% seeding dose in non-neuronal tissues, suggesting that prion-seeding activity exists in non-neural organs, and we suggested that non-neural tissues of 106/g SD50 did not exist the infectivity.
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spelling doaj.art-18982ab983274cdc80ccd6ad61d4e4e72022-12-21T21:56:24ZengElsevierEBioMedicine2352-39642016-10-0112C15015510.1016/j.ebiom.2016.08.033Prion-Seeding Activity Is widely Distributed in Tissues of Sporadic Creutzfeldt-Jakob Disease PatientsHanae Takatsuki, PhD0Takayuki Fuse, PhD1Takehiro Nakagaki, MD, PhD2Tsuyoshi Mori, PhD3Ban Mihara, MD, PhD4Masaki Takao, MD, PhD5Yasushi Iwasaki, MD, PhD6Mari Yoshida, MD, PhD7Shigeo Murayama, MD, PhD8Ryuichiro Atarashi, MD, PhD9Noriyuki Nishida, MD, PhD10Katsuya Satoh, MD, PhD11Department of Molecular Microbiology and Immunology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, JapanDepartment of Molecular Microbiology and Immunology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, JapanDepartment of Molecular Microbiology and Immunology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, JapanDepartment of Infectious Diseases, Faculty of Medicine, University of Miyazaki, Miyazaki, JapanDepartment of Neurology, Institute of Brain and Blood Vessels, Mihara Memorial Hospital, Isesaki, JapanDepartment of Neurology, Institute of Brain and Blood Vessels, Mihara Memorial Hospital, Isesaki, JapanDepartment of Neuropathology, Institute for Medical Science of Aging, Aichi Medical University, Aichi, JapanDepartment of Neuropathology, Institute for Medical Science of Aging, Aichi Medical University, Aichi, JapanDepartment of Neuropathology, Tokyo Metropolitan Geriatric Hospital and Institute of Gerontology, Tokyo, JapanDepartment of Infectious Diseases, Faculty of Medicine, University of Miyazaki, Miyazaki, JapanDepartment of Molecular Microbiology and Immunology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, JapanDepartment of Locomotive Rehabilitation Science, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, JapanHuman prion diseases are neurodegenerative disorders caused by abnormally folded prion proteins in the central nervous system. These proteins can be detected using the quaking-induced conversion assay. Compared with other bioassays, this assay is extremely sensitive and was used in the present study to determine prion distribution in sporadic Creutzfeldt-Jakob disease patients at autopsy. Although infectivity of the sporadic form is thought to be restricted within the central nervous system, results showed that prion-seeding activities reach 106/g from a 50% seeding dose in non-neuronal tissues, suggesting that prion-seeding activity exists in non-neural organs, and we suggested that non-neural tissues of 106/g SD50 did not exist the infectivity.http://www.sciencedirect.com/science/article/pii/S2352396416303887PrionPrion-seeding activitySD50Non-neural tissueCreutzfeldt-Jakob disease
spellingShingle Hanae Takatsuki, PhD
Takayuki Fuse, PhD
Takehiro Nakagaki, MD, PhD
Tsuyoshi Mori, PhD
Ban Mihara, MD, PhD
Masaki Takao, MD, PhD
Yasushi Iwasaki, MD, PhD
Mari Yoshida, MD, PhD
Shigeo Murayama, MD, PhD
Ryuichiro Atarashi, MD, PhD
Noriyuki Nishida, MD, PhD
Katsuya Satoh, MD, PhD
Prion-Seeding Activity Is widely Distributed in Tissues of Sporadic Creutzfeldt-Jakob Disease Patients
EBioMedicine
Prion
Prion-seeding activity
SD50
Non-neural tissue
Creutzfeldt-Jakob disease
title Prion-Seeding Activity Is widely Distributed in Tissues of Sporadic Creutzfeldt-Jakob Disease Patients
title_full Prion-Seeding Activity Is widely Distributed in Tissues of Sporadic Creutzfeldt-Jakob Disease Patients
title_fullStr Prion-Seeding Activity Is widely Distributed in Tissues of Sporadic Creutzfeldt-Jakob Disease Patients
title_full_unstemmed Prion-Seeding Activity Is widely Distributed in Tissues of Sporadic Creutzfeldt-Jakob Disease Patients
title_short Prion-Seeding Activity Is widely Distributed in Tissues of Sporadic Creutzfeldt-Jakob Disease Patients
title_sort prion seeding activity is widely distributed in tissues of sporadic creutzfeldt jakob disease patients
topic Prion
Prion-seeding activity
SD50
Non-neural tissue
Creutzfeldt-Jakob disease
url http://www.sciencedirect.com/science/article/pii/S2352396416303887
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