| First Author | Lindsten K | Year | 2003 |
| Journal | Nat Biotechnol | Volume | 21 |
| Issue | 8 | Pages | 897-902 |
| PubMed ID | 12872133 | Mgi Jnum | J:127085 |
| Mgi Id | MGI:3762780 | Doi | 10.1038/nbt851 |
| Citation | Lindsten K, et al. (2003) A transgenic mouse model of the ubiquitin/proteasome system. Nat Biotechnol 21(8):897-902 |
| abstractText | Impairment of the ubiquitin/proteasome system has been proposed to play a role in neurodegenerative disorders such as Alzheimer and Parkinson diseases. Although recent studies confirmed that some disease-related proteins block proteasomal degradation, and despite the existence of excellent animal models of both diseases, in vivo data about the system are lacking. We have developed a model for in vivo analysis of the ubiquitin/proteasome system by generating mouse strains transgenic for a green fluorescent protein (GFP) reporter carrying a constitutively active degradation signal. Administration of proteasome inhibitors to the transgenic animals resulted in a substantial accumulation of GFP in multiple tissues, confirming the in vivo functionality of the reporter. Moreover, accumulation of the reporter was induced in primary neurons by UBB+1, an aberrant ubiquitin found in Alzheimer disease. These transgenic animals provide a tool for monitoring the status of the ubiquitin/proteasome system in physiologic or pathologic conditions. |
