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D-Glucuronic acid is a metabolite of glucose.1,2 It is formed from glucose in a multi-step process in which uridine diphosphate glucose (UDP-α-D-glucose; Item No. 15602) is dehydrogenated to uridine diphosphate glucuronic acid (uridine-5’-diphosphoglucuronic acid; Item No. 20674), from which D-glucuronic acid can be transferred to a receptor to form glucuronides, further metabolized to ascorbic acid or xylulose, or excreted.1 D-Glucuronic acid is a component of proteoglycans, including heparan sulfate and chondroitin sulfate.3 Levels of D-glucuronic acid are increased in fibroblasts isolated from patients with infantile free sialic acid storage disease (ISSD) or Salla disease, lysosomal storage disorders characterized by truncal ataxia and psychomotor retardation and heptatosplenomegaly and impaired growth, respectively.4
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1. Enzyme levels of glucuronic acid metabolism in the liver, kidney and intestine of normal and fasted rats. Biochem. Pharmacol. 12(6), 565-575 (1963).
2. Glucuronide-
3. Chondroitin sulfate: A key molecule in the brain matrix. Int. J. Biochem. Cell Biol. 44(4), 582-586 (2012).
4. Defective glucuronic acid transport from lysosomes of infantile free sialic acid storage disease fibroblasts. Biochem. J. 268(3), 621-625 (1990).