DOI: 10.1126/science.1980379. Spontaneous Neurodegeneration in Transgenic Mice with Mutant Prion Protein

Spontaneous Neurodegeneration in Transgenic Mice with Mutant Prion Protein

10.1126/science.1980379

Crossref journal-article

American Association for the Advancement of Science (AAAS)

Science (221)

Abstract

Transgenic mice were created to assess genetic linkage between Gerstmann-Sträussler-Scheinker syndrome and a leucine substitution at codon 102 of the human prion protein gene. Spontaneous neurologic disease with spongiform degeneration and gliosis similar to that in mouse scrapie developed at a mean age of 166 days in 35 mice expressing mouse prion protein with the leucine substitution. Thus, many of the clinical and pathological features of Gerstmann-Sträussler-Scheinker syndrome are reproduced in transgenic mice containing a prion protein with a single amino acid substitution, illustrating that a neurodegenerative process similar to a human disease can be genetically modeled in animals.

Bibliography

Hsiao, K. K., Scott, M., Foster, D., Groth, D. F., DeArmond, S. J., & Prusiner, S. B. (1990). Spontaneous Neurodegeneration in Transgenic Mice with Mutant Prion Protein. Science, 250(4987), 1587â1590.

Authors 6

Karen K. Hsiao (first)
Michael Scott (additional)
Dallas Foster (additional)
Darlene F. Groth (additional)
Stephen J. DeArmond (additional)
Stanley B. Prusiner (additional)

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Dates

Type	When
Created	18 years, 10 months ago (Oct. 5, 2006, 6:10 p.m.)
Deposited	1 year, 7 months ago (Jan. 11, 2024, 10:37 p.m.)
Indexed	1 month, 3 weeks ago (July 6, 2025, 1:26 p.m.)
Issued	34 years, 8 months ago (Dec. 14, 1990)
Published	34 years, 8 months ago (Dec. 14, 1990)
Published Print	34 years, 8 months ago (Dec. 14, 1990)

Funders 0

None

BibTeX

@article{Hsiao_1990, title={Spontaneous Neurodegeneration in Transgenic Mice with Mutant Prion Protein}, volume={250}, ISSN={1095-9203}, url={http://dx.doi.org/10.1126/science.1980379}, DOI={10.1126/science.1980379}, number={4987}, journal={Science}, publisher={American Association for the Advancement of Science (AAAS)}, author={Hsiao, Karen K. and Scott, Michael and Foster, Dallas and Groth, Darlene F. and DeArmond, Stephen J. and Prusiner, Stanley B.}, year={1990}, month=dec, pages={1587–1590} }

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