Table 1. Glucosylsphingosine concentration in N370S/N370S hMacs and IVS2+1/L444P and N370S/N370S iMacs.

Glucosylsphingosine concentrations (picomole per microgram of protein) were measured in control and Gaucher disease iMacs and hMacs using LC-MS/MS. hMacs and iMacs were derived from patients with genotype N370S/N370S. iMacs were also derived from a patient with genotype IVS2+1/L444P. Groups were compared using one-way ANOVA with Bonferroni correction. ***P = 0.0017; *P = 0.0368.

Control hMacsN370S/N370S hMacsControl iMacsiMacs IVS2+1/L444PiMacs N370S/N370S
Untreated0.0832 ± 0.0130.2372 ± 0.2***0.047 ± 0.151.121***0.622
+Ghosts and NCGC001887580.0986 ± 0.030.1352 ± 0.020.0700.227*0.417