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Publication : A specific inhibitor of janus kinase-3 increases survival in a transgenic mouse model of amyotrophic lateral sclerosis.

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.
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