化学性质：                                                                                                             

产品描述：                                                                                                            

N-Butyldeoxynojirimycin-d9 (NB-DNJ-d9) is intended for use as an internal standard for the quantification of NB-DNJ by GC- or LC-MS. NB-DNJ is an iminosugar that inhibits UDP-glucose ceramide glucosyltransferase and β-glucosidase 2 (IC50s = 32 and 81 μM, respectively, for the rat recombinant enzymes).1 It also increases the activity of wild-type acid β-glucosidase, as well as the S364R, N370S, V15M, or M123T mutants, in COS-7 cells expressing the human enzymes when used at a concentration of 10 μM.2 NB-DNJ also inhibits HIV-1 and HIV-2 infection of peripheral blood mononuclear cells (PBMCs; IC50s = 282 and 211 μM, respectively).3 Formulations containing NB-DNJ have been used in the treatment of Gaucher disease, an inborn error of metabolism characterized as a lysosomal storage disorder resulting from substantial deficiency of β-glucosidase.

特别提醒：