13C6 Glucosylsphingosine (d18:1)

13C6 Glucosylsphingosine (D18:1) is an isotopically enriched form of 1-β-D-glucosylsphingosine (D18:1)that is intended for use as an internal standard for the quantification of 1-β-D-glucosylsphingosine by GC- or LC-MS. 1-β-D-Glucosylsphingosine is a lysolipid derivative of glucosylcerebroside that decreases activity of glucocerebrosidase in LA-N-2 cells in a dose-dependent manner.

Glucosylsphingosine(Lyso-Gb1) 是一种天然存在的糖基神经酰胺, 是GBA (acid β-glucosidase）的代谢产物, 参与细胞识别、信号转导和细胞间相互作用。Glucopsychosine在某些神经退行性疾病（如 Gaucher病）中积累，通过激活哺乳动物雷帕霉素复合体1（mTORC1）来破坏溶酶体功能,也是一种非竞争性糖脑苷酶 (glucocerebrosidase) 抑制剂。