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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.
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July 1996,
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March 2009,
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K K Hsiao, and
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June 2000,
Neuropathology and applied neurobiology,
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Molecular and cellular biology,
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April 1997,
Protein science : a publication of the Protein Society,
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The Journal of neuroscience : the official journal of the Society for Neuroscience,
K K Hsiao, and
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S B Prusiner
February 2012,
Proceedings of the National Academy of Sciences of the United States of America,
K K Hsiao, and
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D Foster, and
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S B Prusiner
October 1998,
Brain pathology (Zurich, Switzerland),
