Characterization of glycosaminoglycans stored in mucopolysaccharidosis III A: evidence for a generally occuring degradation of heparan sulfate by endoglycosidases.

Abstract

The characterization of intracellularly stored glycosaminoglycans from organs of a patient suffering from mucopolysaccharidosis III A (Sanfilippo A disease) is described. Both heparan sulfate and galactosamine-containing glycosaminoglycans (chondroitin sulfate, dermatan sulfate) are accumulated in the liver, whereas in the other organs (spleen, kidney, heart, cerebrum, cerebellum) heparan sulfate is almost the only glycosaminoglycan stored. It is shown by [3H]NaBH4 reduction and subsequent identification of the 3H-labelled sugar alcohols that heparan sulfate is degraded in all organs by at least two endoglycosidases, an endoglucuronidase and an endoglucosaminidase, to fragments of low molecular weight (Mr approximately 2 000-6 600).

• Authors: A Kindler, U Klein, K von Figura
• Journal: Hoppe-Seyler's Zeitschrift fur physiologische Chemie (Hoppe Seylers Z Physiol Chem) Vol. 358 Issue 11 Pg. 1431-8 (Nov 1977) ISSN: 0018-4888 [Print] Germany
• PMID: 411726 (Publication Type: Journal Article)

Chemical References

• Carbohydrates
• Glycosaminoglycans
• Heparitin Sulfate
• Glycoside Hydrolases

Topics

• Carbohydrates (analysis)
• Glycosaminoglycans (metabolism)
• Glycoside Hydrolases
• Heparitin Sulfate (metabolism)
• Humans
• Mucopolysaccharidoses (metabolism)
• Mucopolysaccharidosis III (metabolism)
• Tissue Distribution