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Science 31 August 1973:
Vol. 181. no. 4102, pp. 866 - 867
DOI: 10.1126/science.181.4102.866
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Articles

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

Jerry N. Thompson 1, Allen C. Stoolmiller 1, Reuben Matalon 1, and Albert Dorfman 1

1 Departments of Pediatrics and Biochemistry, Joseph P. Kennedy, Jr., Mental Retardation Research Center, La Rabida-University of Chicago Institute, Pritzker School of Medicine, University of Chicago, Chicago, Illinois 60637

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-beta-hexosaminidase A is responsible for degradation of the oligosaccharide substrate.

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Science. ISSN 0036-8075 (print), 1095-9203 (online)