Investigation of the Impact of Dual Atp10B- and Gba1-Deficiency on glycolipds in a Murine Model

The combined effects of GBA1 and ATP10B deficiency on lipid accumulation were investigated in a murine model. GCase activity can be pharmacologically inhibited in vivo by administration of the GCase inhibitor conduritol-β-epoxide (CBE) via intraperitoneal (IP) injection. Three-month-old Atp10b knockout and wild-type mice were administered CBE at a dose of 100 mg/kg body weight via IP injection for 28 consecutive days. Glucosylceramide and glucosylsphingosine levels in brain tissues were subsequently analyzed by LC/MS. Relative quantification was performed using the peak area ratio of each analyte to its corresponding internal standard: d7-glucosylsphingosine for glucosylsphingosine and d5-C18:0 glucosylceramide for glucosylceramide.