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- W2331746009 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)." @default.
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- W2331746009 date "1977-01-01" @default.
- W2331746009 modified "2023-09-26" @default.
- W2331746009 title "Characterization of Glycosaminoglycans Stored in Mucopolysaccharidosis III A: Evidence for a Generally Occuring Degradation of Heparan Sulfate by Endoglycosidases" @default.
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- W2331746009 doi "https://doi.org/10.1515/bchm2.1977.358.2.1431" @default.
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