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 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.
Department of Molecular Microbiology and Immunology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan.
Hanae
H
Takatsuki
Department of Molecular Microbiology and Immunology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan.
Takayuki
T
Fuse
Department of Molecular Microbiology and Immunology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan.
Takehiro
T
Nakagaki
Department of Infectious Diseases, Faculty of Medicine, University of Miyazaki, Miyazaki, Japan.
Tsuyoshi
T
Mori
Department of Neurology, Institute of Brain and Blood Vessels, Mihara Memorial Hospital, Isesaki, Japan.
Ban
B
Mihara
Department of Neurology, Institute of Brain and Blood Vessels, Mihara Memorial Hospital, Isesaki, Japan; Department of Neurology International Medical Center, Saitama Medical University, Saitama, Japan.
Masaki
M
Takao
Department of Neuropathology, Institute for Medical Science of Aging, Aichi Medical University, Aichi, Japan.
Yasushi
Y
Iwasaki
Department of Neuropathology, Institute for Medical Science of Aging, Aichi Medical University, Aichi, Japan.
Mari
M
Yoshida
Department of Neuropathology, Tokyo Metropolitan Geriatric Hospital and Institute of Gerontology, Tokyo, Japan.
Shigeo
S
Murayama
Department of Infectious Diseases, Faculty of Medicine, University of Miyazaki, Miyazaki, Japan.
Ryuichiro
R
Atarashi
Department of Molecular Microbiology and Immunology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan.
Noriyuki
N
Nishida
Department of Locomotive Rehabilitation Science, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan. Electronic address: satoh-prion@nagasaki-u.ac.jp.
Katsuya
K
Satoh
D000368:Aged; D000818:Animals; D001344:Autopsy; D001921:Brain / Q000378:metabolism; D016022:Case-Control Studies; D007562:Creutzfeldt-Jakob Syndrome / Q000175:diagnosis / Q000235:genetics / Q000378:metabolism* / Q000473:pathology*; D004195:Disease Models, Animal; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D051379:Mice; D008875:Middle Aged; D009928:Organ Specificity; D000072002:Prion Proteins / Q000235:genetics / Q000378:metabolism*
D000072002:Prion Proteins
Creutzfeldt-Jakob disease; Non-neural tissue; Prion; Prion-seeding activity; SD50