Abstract
The causative agent of transmissible spongiform encephalopathies, prion, is thought to be composed mainly of abnormal isoform of prion protein (PrPSc). Although prion is devoid of agent-specific nucleic acid, there exist prion strains that are characterized biologically. Distribution of neuropathological lesions is one of the phenotyes of prion strains, however, there are only a few reports that addressed the linkage of the clinical manifestations to neuropathological lesions. In this study, we compared the biological, biochemical, and neuropathological differences among three mouse adapted-scrapie strains, G1, I3/I5, and Obihiro. G1 exhibited longer incubation periods (∼330 days) than others in mice carrying PrPA/A allotype. Eighty percent of G1 strain-infected ICR mice showed severe obesity and polyuria. Diffuse deposition of PrPSc was widespread in cerebral cortex, hippocampus of I3/I5 and Obihiro strain-infected mice, while in G1 strain-infected mice, deposition of PrPSc was rather restricted in the thalamus and hypothalamus and large PrP amyloid plaques were observed in cerebral cortex. PrPSc of three strains could also be distinguished in combination of relative proteinase K resistance and glycoform patterns. Serum concentration insulin and leptin levels were remarkable high in G1-infected ICR mice with obesity, suggesting endocrinopathy and/or carbohydrate metabolism failure. PrPSc-deposition and neuronal vacuolation were observed the regions in hypothalamus including suprachiasmatic nucleus, supraoptic nucleus and paraventricular nucleus. Kinetic analysis indicated that vasopressin-positive cells in these nuclei decreased along with the progression of the neuronal degeneration. In contrast, vasopressin-positive cells in these nuclei were detected even at the terminal stage of G1-infected C57BL/J6 mice that did not exhibit polyuria. These results suggest that G1-affected ICR mice developed hypothalamic diabetes insipidus. The particular characteristics of G1 strain would be useful for further analyzing the target cell specificity of prion and pathogenesis of prion diseases.
Keywords
- Prion Disease
- Supraoptic Nucleus
- Transmissible Spongiform Encephalopathy
- Serum Concentration Insulin
- Longe Incubation Period
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© 2005 Springer-Verlag Tokyo
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Horiuchi, M., Tamura, Yk., Furuoka, H. (2005). Comparative analyses of three mouse-adapted scrapie strains G1, Obihiro, and I3/I5 and pathogenesis of G1 strain-induced polyuria in ICR mice. In: Kitamoto, T. (eds) Prions. Springer, Tokyo. https://doi.org/10.1007/4-431-29402-3_20
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DOI: https://doi.org/10.1007/4-431-29402-3_20
Publisher Name: Springer, Tokyo
Print ISBN: 978-4-431-25539-0
Online ISBN: 978-4-431-29402-3
eBook Packages: MedicineMedicine (R0)