Description
An eIF2B activator (EC50 = 33 nM in a reporter assay); enhances eIF2B activity in primary fibroblasts isolated from R191H mouse embryos (EC50 = 7.3 nM); normalizes body weight gain and prevents the development of motor deficits in R191H mice at 30 mg/kg; prevents activation of the integrated stress response and myelin loss in the same model