Figure 6. Metabolic and catabolic pathway enzymes for Gb3 synthesis.
A part of Gb3 synthesis pathway is shown. From lactosylceramide (LacCer) to Gb3, alpha 1, 4-galactosyltransferase (EC 2.4.1.228) adds a galactose to LacCer to produce Gb3. Likewise, UDP-GalNAc: beta 1,3-galactosaminyltransferase (EC 2.4.1.79) works on Gb3 to make Gb4. In the catabolic pathway, beta-hexosaminidase (EC 3.2.1.52) degrades Gb4 to Gb3, and alpha-galactosidase (EC 3.2.1.22) makes LacCer from Gb3.