UXS1

Catalyzes the NAD-dependent decarboxylation of UDP-glucuronic acid to UDP-xylose. Necessary for the biosynthesis of the core tetrasaccharide in glycosaminoglycan biosynthesis. Catalyzes the synthesis of UDP-xylose in two steps: the hydroxyl group of UDP-glucuronic acid is first oxidized using an enzyme-bound NAD(+) as electron acceptor, favoring the decarboxylation yielding a UDP-4-ketoxylose reaction intermediate and a reduced cofactor NADH bound to the catalytic pocket. In the second step, the 4-keto group is reduced resulting in UDP-xylose and the restoration of the enzyme to its NAD(+)-bound form..

Function from UniProtKB (Q8NBZ7), read 2026-09-23. CC BY 4.0.