Degradation of keratan sulfate by ß-N-acetylhexosaminidases in GM2-gangliosidosis
- 23 April 2008
- journal article
- research article
- Published by Wiley in Clinical Genetics
- Vol. 21 (3) , 196-202
- https://doi.org/10.1111/j.1399-0004.1982.tb00963.x
Abstract
A new substrate was prepared from a keratan sulfate-derived-oligosaccharide (2-acetamido-2-deoxyglucosyl-(1-3)-[1-3H] Galactitol), which is necessary to measure .beta.-N-acetylhexosaminidase activity. This substrate was prepared from a cornea keratan sulfate by digestion with endo-.beta.-galactosidase, followed by isolation of disaccharide on gel filtration chromatography and chemical desulfation. Using this substrate, a striking deficiency of .beta.-N-acetylhexosaminidase activity was found in the skin fibroblasts of patients with Sandhoff disease but not in Tay-Sachs disease. Both .beta.-N-acetyl-hexosaminidase A and B contributed to the catabolism of keratan sulfate.Keywords
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