Chemical structure and mammalian glycosphingolipid (GSL) biosynthetic pathway. (A) Chemical structure of a typical mammalian GSL, globotriaosylceramide (Gb3Cer), shown as an example. (B) Arrows and blue font indicate biosynthetic pathway and catalytic enzymes, respectively. Abbreviations: GlcCer, Glcβ1,1Cer; GalCer, Galβ1,1Cer; LacCer, Galβ1,4Glcβ1,1Cer; GM3, Siaα2,3Galβ1,4Glcβ1,1Cer; UGCG, UDP-glucose ceramide glucosyltransferase; UGT8, UDP glycosyltransferase 8; GAL3ST1, galactose-3-O-sulfotransferase 1; B4GALT5/6, β-1,4-galactosyltransferase 5/6; B3GNT5, UDP-GlcNAc: βGal β-1,3-N-acetylglucosaminyltransferase 5; A4GALT, α-1,4-galactosyltransferase; B4GALNT1, β-1,4-N-acetyl-galactosaminyltransferase 1; ST3GAL5, ST3 β-galactoside α-2,3-sialyltransferase 5; ST8SIA1, ST8 α-N-acetyl-neuraminide α-2,8-sialyltransferase 1.