Article
Spontaneous neurodegeneration in transgenic mice with mutant prion protein.
Science (New York, N.Y.) - 14 Dec 1990
Hsiao K K, Scott M, Foster D, Groth D F, DeArmond S J, Prusiner S B
Abstract excerpt
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...
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