| First Author | Hsiao KK | Year | 1990 |
| Journal | Science | Volume | 250 |
| Issue | 4987 | Pages | 1587-90 |
| PubMed ID | 1980379 | Mgi Jnum | J:127373 |
| Mgi Id | MGI:3763660 | Doi | 10.1126/science.1980379 |
| Citation | Hsiao KK, et al. (1990) Spontaneous neurodegeneration in transgenic mice with mutant prion protein. Science 250(4987):1587-90 |
| abstractText | Transgenic mice were created to assess genetic linkage between Gerstmann-Straussler-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-Straussler-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. |
