First Author | Trieu VN | Year | 2000 |
Journal | Biochem Biophys Res Commun | Volume | 267 |
Issue | 1 | Pages | 22-5 |
PubMed ID | 10623568 | Mgi Jnum | J:59400 |
Mgi Id | MGI:1351539 | Doi | 10.1006/bbrc.1999.1905 |
Citation | Trieu VN, et al. (2000) A specific inhibitor of janus kinase-3 increases survival in a transgenic mouse model of amyotrophic lateral sclerosis. Biochem Biophys Res Commun 267(1):22-5 |
abstractText | Amyotrophic lateral sclerosis (ALS) is a progressive, fatal neurodegenerative disorder involving the motor neurons of cortex, brain stem, and spinal cord. About 10% of all ALS patients are familial cases (FALS), of which 20% have mutations in the Cu, Zn-superoxide dismutase (SOD1) gene. The murine model for FALS, which overexpresses a FALS variant of the SOD1 gene, exhibits progressive limbic paralysis followed by death. Treatment of FALS mice with WHI-P131, a specific inhibitor of Janus kinase 3 (JAK3), increased survival by more than two months, suggesting that specific inhibitors of JAK3 may be useful in the treatment of human ALS. These results uniquely establish JAK3 as a novel molecular target for the treatment of FALS. Copyright 2000 Academic Press. |