产品描述:
2BAct is an activator of eukaryotic translation initiation factor 2B (eIF2B; EC50 = 33 nM in a reporter assay).{69170} It enhances eIF2B activity in primary fibroblasts isolated from Eif2b5R191H mutant (R191H) mouse embryos (EC50 = 7.3 nM), a model of vanishing white matter (VWM) leukodystrophy. In vivo, 2BAct (30 mg/kg) normalizes body weight gain and prevents the development of motor deficits in R191H mice. It also prevents activation of the integrated stress response and myelin loss in the same model.