Deep phenotyping of SH-Sy5y cells exposed to glucosylsphingosine by LC-MS

SH-Sy5y cells were exposed to glucosylsphingosine (GlcSph) at two concentrations observed in moderate (20 ng/mL) and severe (200 ng/mL) Gaucher disease phenotypes and subjected to label free proteomics to identify cellular pathways and molecules affected by GlcSph. Lyso-Gb3, a lipid accumulating in Fabry disease, which exhibits no nerodegeneration, was used as a disease control. DMSO was used as a vehicle control.Proteomics analysis demonstrated a negative effect of GlcSph on cell metabolism, particularly affecting the TCA cycle, mitochondrial function, glycolysis and protein ubiquitination.