| First Author | Stöhr J | Year | 2011 |
| Journal | Proc Natl Acad Sci U S A | Volume | 108 |
| Issue | 52 | Pages | 21223-8 |
| PubMed ID | 22160704 | Mgi Jnum | J:180145 |
| Mgi Id | MGI:5305522 | Doi | 10.1073/pnas.1117827108 |
| Citation | Stohr J, et al. (2011) Spontaneous generation of anchorless prions in transgenic mice. Proc Natl Acad Sci U S A 108(52):21223-8 |
| abstractText | Some prion protein mutations create anchorless molecules that cause Gerstmann-Straussler-Scheinker (GSS) disease. To model GSS, we generated transgenic mice expressing cellular prion protein (PrP(C)) lacking the glycosylphosphatidyl inositol (GPI) anchor, denoted PrP(DeltaGPI). Mice overexpressing PrP(DeltaGPI) developed a late-onset, spontaneous neurologic dysfunction characterized by widespread amyloid deposition in the brain and the presence of a short protease-resistant PrP fragment similar to those found in GSS patients. In Tg(PrP,DeltaGPI) mice, disease onset could be accelerated either by inoculation with brain homogenate prepared from spontaneously ill animals or by coexpression of membrane-anchored, full-length PrP(C). In contrast, coexpression of N-terminally truncated PrP(Delta23-88) did not affect disease progression. Remarkably, disease from ill Tg(PrP,DeltaGPI) mice transmitted to mice expressing wild-type PrP(C), indicating the spontaneous generation of prions. |
