Fig. 1

Molecular structure of disialoganglioside GD2 and the GSL biosynthesis pathway with focus on ganglioside biosynthesis. A) Exemplarily, GD2’s head group consists of \(\beta\)-Glucose (Glc), linked to the ceramide backbone, \(\beta\)-galactose (Gal), N-acetyl-D-galactosamin (GalNAc), and two N-acetylneuraminic acid molecules (NeuNAc). The sphingoid base and the fatty acid of the ceramide anchor are respectively blue and red. B) The GSL biosynthesis pathway starts with the synthesis of glucosylceramide (GlcCer) from ceramide and the further synthesis from GlcCer to lactosylceramide (LacCer). The addition of sulfate groups to LacCer leads to lactosylceramide sulfate (SM3). From LacCer the biosynthesis pathway splits into three subseries, the globo series, the lacto- and neolacto series, and the ganglio series. The ganglio-series consists of four subseries, the 0-, a-, b-, and c-series. The genes coding for the enzymes responsible for the ganglioside biosynthesis are B4GALNT1, B3GALT4, ST3GAL2/3/5, and ST8SIA1/3/5. Figure elements created with Biorender