N-Acetyl-β-hexosaminidase: Role in the Degradation of Glycosaminoglycans

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Science  31 Aug 1973:
Vol. 181, Issue 4102, pp. 866-867
DOI: 10.1126/science.181.4102.866


Extracts of cultured normal human skin fibroblasts released radioactivity from a 14C-labeled heptasaccharide prepared by addition of [14C]N-acetylgalactosamine to the nonreducing terminus of a hexasaccharide derived from chondroitin 4-sulfate whereas fibroblast extracts from patients with Tay-Sachs and Sandhoff-Jatzkewitz diseases did not. The results suggest that N-acetyl-β-hexosaminidase A is responsible for degradation of the oligosaccharide substrate.