| First Author | Puttaparthi K | Year | 2007 |
| Journal | Exp Neurol | Volume | 206 |
| Issue | 1 | Pages | 53-8 |
| PubMed ID | 17482163 | Mgi Jnum | J:141580 |
| Mgi Id | MGI:3818815 | Doi | 10.1016/j.expneurol.2007.03.024 |
| Citation | Puttaparthi K, et al. (2007) Assessing the role of immuno-proteasomes in a mouse model of familial ALS. Exp Neurol 206(1):53-8 |
| abstractText | The accumulation of protein aggregates is thought to be an important component in the pathogenesis of mutant SOD1-induced disease. Mutant SOD1 aggregates appear to be cleared by proteasomes, at least in vitro, suggesting a potentially important role for proteasome degradation pathways in vivo. G93A SOD1 transgenic mice show an increase in proteasome activity and induction of immuno-proteasome subunits within spinal cord as they develop neurological symptoms. To determine what role immuno-proteasomes may have in mutant SOD1-induced disease, we crossed G93A SOD1 transgenic mice with LMP2-/- mice to obtain G93A SOD1 mice lacking the LMP2 immuno-proteasome subunit. G93A SOD1/LMP2-/- mice show significant reductions in proteasome function within spinal cord compared to G93A SOD1 mice. However, G93A SOD1/LMP2-/- mice show no change in motor function decline, or survival compared to G93A SOD1 mice. These results indicate that the loss of immuno-proteasome function in vivo does not significantly alter mutant SOD1-induced disease. |
