TGDS

UDP-glucose 4,6-dehydratase that converts UDP-glucose into UDP-4-keto-6-deoxyglucose, and which is required for glycosaminoglycan biosynthesis and skeletal development. UDP-4-keto-6-deoxyglucose is a mimic of the reaction intermediate of UXS1 and acts as an enzyme-rescue metabolite to promote the completion of UXS1 catalytic cycle when NAD(+) levels are low. Under low NAD(+) conditions, UXS1 forms an inactive UDP-4-ketoxylose intermediate bound to NADH, impairing the synthesis of specific glycans that are essential for skeletal development. UDP-4-keto-6-deoxyglucose is used by the inactive NADH-bound UXS1 to produce UDP-6-deoxyglucose and NAD(+) within the catalytic pocket of UXS1, regenerating the essential cofactor NAD(+)..

Function from UniProtKB (O95455), read 2026-09-23. CC BY 4.0.