N-(n-Butyl)deoxygalactonojirimycin

N-(n-Butyl)deoxygalactonojirimycin (NB-DGJ) is an inhibitor of the glycolipid biosynthesis enzyme glucosylceramide synthase and the glycolipid catabolic enzymes glucocerebrosidase (GBA) and beta-glucosidase 2 (GBA2). It also inhibits acid alpha-glucosidase, sucrase, maltase, and lactase. NB-DGJ (0.5-500 µM) reduces glycolipid levels in HL-60 and WEHI-3B cells. It decreases brain ganglioside levels in a Glb1-/- neonatal mouse model of GM1 gangliosidosis when administered intraperitoneally at doses of 600 and 1,200 mg/kg four times per day for three days. NB-DGJ (1-1,200 mg/kg per day for 35 days) increases testicular glucosylceramide levels in mice. NB-DGJ induces abnormal spermatid and acrosome formation, as well as reduces motility, in isolated mouse epididymal sperm when administered at doses of 15, 25, and 50 mg/kg per day. It also induces infertility, an effect that is reversed by withdrawal, in the same model.Formal Name: 1-butyl-2R-(hydroxymethyl)-3S,4R,5S-piperidinetriol. CAS Number: 141206-42-0. Synonyms: Lucerastat, NBDGJ. Molecular Formula: C10H21NO4. Formula Weight: 219.3. Purity: >95%. Formulation: (Request formulation change), A crystalline solid. Solubility: DMF: 20 mg/mL, DMSO: 30 mg/mL, Ethanol: 5 mg/mL, PBS (pH 7.2): 10 mg/mL. SMILES: OC[C@H]1N(CCCC)C[C@H](O)[C@@H](O)[C@H]1O. InChi Code: InChI=1S/C10H21NO4/c1-2-3-4-11-5-8(13)10(15)9(14)7(11)6-12/h7-10,12-15H,2-6H2,1H3/t7-,8+,9+,10-/m1/s1. InChi Key: UQRORFVVSGFNRO-XFWSIPNHSA-N.