| First Author | Beaulieu JM | Year | 1999 |
| Journal | J Cell Biol | Volume | 147 |
| Issue | 3 | Pages | 531-44 |
| PubMed ID | 15132161 | Mgi Jnum | J:58388 |
| Mgi Id | MGI:1347434 | Doi | 10.1083/jcb.147.3.531 |
| Citation | Beaulieu JM, et al. (1999) Late onset death of motor neurons in mice overexpressing wild-type peripherin. J Cell Biol 147(3):531-44 |
| abstractText | Peripherin, a type III intermediate filament (IF) protein, upregulated by injury and inflammatory cytokines, is a component of IF inclusion bodies associated with degenerating motor neurons in sporadic amyotrophic lateral sclerosis (ALS). We report here that sustained overexpression of wild-type peripherin in mice provokes massive and selective degeneration of motor axons during aging. Remarkably, the onset of peripherin-mediated disease was precipitated by a deficiency of neurofilament light (NF-L) protein, a phenomenon associated with sporadic ALS. In NF-L null mice, the overexpression of peripherin led to early- onset formation of IF inclusions and to the selective death of spinal motor neurons at 6 mo of age. We also report the formation of similar peripherin inclusions in presymptomatic transgenic mice expressing a mutant form of superoxide dismutase linked to ALS. Taken together, these results suggest that IF inclusions containing peripherin may play a contributory role in motor neuron disease. |
