Extraneural manifestations of prion infection in GPI-anchorless transgenic mice

Journal Article · Tue Mar 01 00:00:00 EST 2011 · Virology

DOI:https://doi.org/10.1016/j.virol.2010.12.012· OSTI ID:21486924

Paulsson, Johan F ^[1]; Cruite, Justin; Andaya, Abegail A; Trifilo, Matthew J ^[2]

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1. Department of Chemistry, Scripps Research Institute, 10550 N. Torrey Pines Rd., La Jolla, CA 92037 (United States)
2. Viral Immunobiology Laboratory, Department of Immunology and Microbial Science, Scripps Research Institute, 10550 N. Torrey Pines Rd., La Jolla, CA 92037 (United States)

Earlier studies indicated that transgenic (tg) mice engineered to express prion protein (PrP) lacking the glycophosphatidylinositol (GPI{sup -/-}) membrane anchor formed abnormal proteinase-resistant prion (PrPsc) amyloid deposits in their brains and hearts when infected with the RML strain of murine scrapie. In contrast, RML scrapie infection of normal mice with a GPI-anchored PrP did not deposit amyloid with PrPsc in the brain or the heart. Here we report that scrapie-infected GPI{sup -/-} PrP tg mice also deposit PrP and transmissible infectious material in the gut, kidneys, and islets of Langerhans. Similar to previously reported amyloid deposits in the brain and heart, amyloid deposits were found in the gut; however, no amyloid deposited in the islets. By high-resolution electron microscopy, we show PrP is located primarily in {alpha} cells and also {beta} cells. Islets contain abundant insulin and there is no abnormality in glucose metabolism in infected GPI{sup -/-} PrP tg mice.

• OSTI ID: 21486924
• Journal Information: Virology, Vol. 411, Issue 1; Other Information: DOI: 10.1016/j.virol.2010.12.012; PII: S0042-6822(10)00766-X; Copyright (c) 2010 Elsevier Science B.V., Amsterdam, The Netherlands, All rights reserved.; ISSN 0042-6822
• Country of Publication: United States
• Language: English

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60 APPLIED LIFE SCIENCES
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ELECTRON MICROSCOPY
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METABOLISM
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TRANSGENIC MICE
ALDEHYDES
ANIMALS
BODY
CARBOHYDRATES
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CENTRAL NERVOUS SYSTEM
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MAMMALS
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