Chemical structure of urinary dermatan sulfate excreted by a patient with the Hunter syndrome.

Abstract

The chemical structure of dermatan sulfate (DS) in the urine of a patient the Hunter syndrome was studied through the analysis of disaccharide units which were derived from the urinary DS by digestion with chondroitinase ABC and separated on a Dowex 1 column. The DS was basically composed of repeating disaccharide units of iduronyl N-acetylgalactosamine 4-sulfate. About 90% of the excess sulfate were linked to the iduronate residues as an additional sulfate group in the unit. N-Acetylgalactosamine 6-sulfate and N-acetylgalactosamine 4,6-disulfate residues were minor components. No non-sulfated disaccharide unit was detected in the digestion products. Only sulfoiduronate residue was found as the non-reducing terminal sugar of the DS molecule, consistent with the lack of iduronosulfate sulfatase in this disease.

• Authors: A Kimura, S Hayashi, K Tsurumi
• Journal: The Tohoku journal of experimental medicine (Tohoku J Exp Med) Vol. 131 Issue 3 Pg. 241-7 (Jul 1980) ISSN: 0040-8727 [Print] Japan
• PMID: 6774445 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Disaccharides
• Dermatan Sulfate
• Chondroitin
• Chondroitin Sulfates
• Chondroitinases and Chondroitin Lyases
• Acetylgalactosamine

Topics

• Acetylgalactosamine (analysis)
• Animals
• Chemical Phenomena
• Chemistry
• Child, Preschool
• Chondroitin (analogs & derivatives)
• Chondroitin Sulfates (urine)
• Chondroitinases and Chondroitin Lyases (pharmacology)
• Dermatan Sulfate (analysis, urine)
• Disaccharides (analysis)
• Humans
• Male
• Mucopolysaccharidosis II (urine)
• Skin (analysis)
• Swine