| First Author | Yamamoto A | Year | 2000 |
| Journal | Cell | Volume | 101 |
| Issue | 1 | Pages | 57-66 |
| PubMed ID | 10778856 | Mgi Jnum | J:61490 |
| Mgi Id | MGI:1355044 | Doi | 10.1016/S0092-8674(00)80623-6 |
| Citation | Yamamoto A, et al. (2000) Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease. Cell 101(1):57-66 |
| abstractText | Neurodegenerative disorders like Huntington's disease (HD) are characterized by progressive and putative irreversible clinical and neuropathological symptoms, including neuronal protein aggregates. Conditional transgenic models of neurodegenerative diseases therefore could be a powerful means to explore the relationship between mutant protein expression and progression of the disease. We have created a conditional model of HD by using the tet-regulatable system. Mice expressing a mutated huntingtin fragment demonstrate neuronal inclusions, characteristic neuropathology, and progressive motor dysfunction. Blockade of expression in symptomatic mice leads to a disappearance of inclusions and an amelioration of the behavioral phenotype. We thus demonstrate that a continuous influx of the mutant protein is required to maintain inclusions and symptoms, raising the possibility that HD may be reversible. |
