Spontaneous neurodegeneration in transgenic mice with mutant prion protein

Karen K. Hsiao, Michael Scott, Dallas Foster, Darlene F. Groth, Stephen J. Dearmond, Stanley B. Prusiner

Research output: Contribution to journalArticlepeer-review

443 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)1587-1590
Number of pages4
JournalScience
Volume250
Issue number4987
DOIs
StatePublished - Dec 14 1990

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