N-acetyl-beta-hexosaminidase: role in the degradation of glycosaminoglycans.
Extracts of cultured normal human skin fibroblasts released radioactivity from a (14)C-labeled heptasaccharide prepared by addition of [(14)C]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.
D002239:Carbohydrate Metabolism, Inborn Errors / Q000201:enzymology; D002247:Carbon Isotopes; D002478:Cells, Cultured; D003374:Coumarins / Q000096:biosynthesis; D005347:Fibroblasts; D005688:Galactosamine / Q000096:biosynthesis; D006025:Glycosaminoglycans / Q000378:metabolism*; D006596:Hexosaminidases / Q000378:metabolism*; D006801:Humans; D008607:Intellectual Disability / Q000201:enzymology; D008064:Lipidoses / Q000201:enzymology; D009083:Mucopolysaccharidoses / Q000201:enzymology / Q000235:genetics; D009844:Oligosaccharides / Q000378:metabolism; D012174:Retinitis Pigmentosa / Q000201:enzymology
D002247:Carbon Isotopes; D003374:Coumarins; D006025:Glycosaminoglycans; D009844:Oligosaccharides; D005688:Galactosamine; D006596:Hexosaminidases