biological and biochemical characterization of mice expressing prion protein devoid of the octapeptide repeat region after infection with prions生物和生化特性的老鼠朊蛋白表达缺乏感染朊病毒后八肽重复区域.pdf
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Biological and Biochemical Characterization of Mice
Expressing Prion Protein Devoid of the Octapeptide
Repeat Region after Infection with Prions
1,2 3 1 4 1
Yoshitaka Yamaguchi , Hironori Miyata , Keiji Uchiyama , Akira Ootsuyama , Sachiko Inubushi ,
1 1 2 1,2
Tsuyoshi Mori , Naomi Muramatsu , Shigeru Katamine , Suehiro Sakaguchi *
1 Division of Molecular Neurobiology, The Institute for Enzyme Research (KOSOKEN), The University of Tokushima, Tokushima, Japan, 2 Department of Molecular
Microbiology and Immunology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan, 3 Animal Research Center, School of Medicine, University of
Occupational and Environmental Health, Kitakyushu, Japan, 4 Department of Radiation Biology and Health, School of Medicine, University of Occupational and
Environmental Health, Kitakyushu, Japan
Abstract
Accumulating lines of evidence indicate that the N-terminal domain of prion protein (PrP) is involved in prion susceptibility
in mice. In this study, to investigate the role of the octapeptide repeat (OR) region alone in the N-terminal domain for the
susceptibility and pathogenesis of prion disease, we intracerebrally inoculated RML scrapie prions into tg(PrPDOR)/Prnp0/
0 mice, which express mouse PrP missing only the OR region on the PrP-null background. Incubation times of these mice
were not extended. Protease-resistant PrPDOR, or PrPScDOR, was easily detectable but lower in the brains of these mice,
compared to that in control wild-type mice. Consistently, prion titers were slightly lower and astrogliosis was m
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