Urinary Excretion of Disulfated Disaccharides in Hunter Syndrome: Correction by Infusion of a Serum Fraction
- 1 March 1973
- journal article
- research article
- Published by Taylor & Francis in Analytical Letters
- Vol. 6 (3) , 225-233
- https://doi.org/10.1080/00032717308062201
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.Keywords
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