N-Butyldeoxynojirimycin (hydrochloride)

NB-DNJ is an iminosugar that inhibits UDP-glucose ceramide glucosyltransferase and beta-glucosidase 2 (IC50s = 32 and 81 µM, respectively, for the rat recombinant enzymes). It also increases the activity of wild-type acid beta-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. NB-DNJ also inhibits HIV-1 and HIV-2 infection of peripheral blood mononuclear cells (PBMCs, IC50s = 282 and 211 µM, respectively). 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 beta-glucosidase.Formal Name: 1-butyl-2R-(hydroxymethyl)-3R,4R,5S-piperidinetriol, monohydrochloride. CAS Number: 210110-90-0. Synonyms: Miglustat, NB-DNJ. Molecular Formula: C10H21NO4 . HCl. Formula Weight: 255.7. Purity: >98%. Formulation: (Request formulation change), A crystalline solid. Solubility: DMSO: 10 mg/ml, PBS (pH 7.2): 1 mg/ml. SMILES: OC[C@H]1N(CCCC)C[C@H](O)[C@@H](O)[C@@H]1O.Cl. InChi Code: InChI=1S/C10H21NO4.ClH/c1-2-3-4-11-5-8(13)10(15)9(14)7(11)6-12,/h7-10,12-15H,2-6H2,1H3,1H/t7-,8+,9-,10-,/m1./s1. InChi Key: QPAFAUYWVZMWPR-ZSOUGHPYSA-N.