GDP-L-fucose synthase is an enzyme that in humans is encoded by the GFUS gene (previously TSTA3). As its name suggests, the GFUS protein functions as a GDP-L-fucose synthase. As part of this, it binds to NADP(H). It catalyzes the two-step epimerase and the reductase reactions in GDP-D-mannose metabolism, converting GDP-4-keto-6-D-deoxymannose to GDP-L-fucose. GDP-L-fucose is the substrate of several fucosyltransferases involved in the expression of many glycoconjugates, including blood group ABH antigens and developmental adhesion antigens. Mutations in this gene may cause leukocyte adhesion deficiency, type II.
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Further reading
External links PDBe-KB provides an overview of all the structure information available in the PDB for Human GDP-L-fucose synthase






