Urinary Excretion of Disulfated Disaccharides in Hunter Syndrome: Correction by Infusion of a Serum Fraction

Abstract

The presence of disulfated disaccharides containing iduronic acid has been demonstrated in the urine of a Hunter patient by gel filtration of the low molecular weight urinary mucopolysaccharides. Intravenous infusion of a purified fraction from normal human serum causes disappearance of these metabolites which probably represent the substrate of the enzyme deficient in the disease, a sulfatase specific for an ester group situated on position 2 or 3 of the iduronic acid residues of dermatan sulfate.