N-(n-Butyl)deoxygalactonojirim

葡萄糖神经酰胺合酶、GBA、GBA2、酸性α-葡萄糖苷酶、蔗糖酶、麦芽糖酶和乳糖酶的抑制剂；将HL-60和WEHI-3B细胞中的糖脂水平从0.5降低到500µM；每天四次600和1,200mg/kg连续三天，降低Glb1-/-新生小鼠GM1神经节苷脂沉积症模型中的脑神经节苷脂水平；每天1-1,200mg/kg连续35天，增加小鼠睾丸葡萄糖神经酰胺水平；每天15、25和50mg/kg可诱导小鼠附睾分离精子异常精子细胞和顶体形成，并降低精子活力；在同一模型中诱导不育，停药可逆转这一效应。An inhibitor of glucosylceramide synthase, GBA, GBA2, acid α-glucosidase, sucrase, maltase, and lactase; reduces glycolipid levels in HL-60 and WEHI-3B cells from 0.5-500 µM; decreases brain ganglioside levels in a Glb1-/-neonatal mouse model of GM1 gangliosidosis at 600 and 1,200 mg/kg four times per day for three days; increases testicular glucosylceramide levels in mice from 1-1,200 mg/kg per day for 35 days; induces abnormal spermatid and acrosome formation, as well as reduces motility, in isolated mouse epididymal sperm at 15, 25, and 50 mg/kg per day; induces infertility, an effect that is reversed by withdrawal, in the same model.