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 is dehydrogenated to uridine diphosphate glucuronic acid , 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
1.Miettinen, T.A., and Leskinen, E.Enzyme levels of glucuronic acid metabolism in the liver, kidney and intestine of normal and fasted ratsBiochem. Pharmacol.12(6)565-575(1963) 2.Dutton, G.J., and Storey, I.D.E.Glucuronide-forming enzymes: UDPglucuronic acid + R·OH → UDP + R·O· glucuronic acidMethods in Enzymology5159-164(1962) 3.Kwok, J.C.F., Warren, P., and Fawcett, J.W.Chondroitin sulfate: A key molecule in the brain matrixInt. J. Biochem. Cell Biol.44(4)582-586(2012) 4.Blom, H.J., Andersson, H.C., Seppala, R., et al.Defective glucuronic acid transport from lysosomes of infantile free sialic acid storage disease fibroblastsBiochem. J.268(3)621-625(1990)