Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
3
pubmed:dateCreated
1982-9-24
pubmed:abstractText
We have prepared a new substrate 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, we found that a striking deficiency of beta-N-acetylhexosaminidase activity was present in the skin fibroblasts of patients with Sandhoff disease but not in Tay-Sachs disease. Both beta-N-acetyl-hexosaminidase A & H contributed to the catabolism of keratan sulfate.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Mar
pubmed:issn
0009-9163
pubmed:author
pubmed:issnType
Print
pubmed:volume
21
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
196-202
pubmed:dateRevised
2007-11-15
pubmed:meshHeading
pubmed:year
1982
pubmed:articleTitle
Degradation of keratan sulfate by beta-N-acetylhexosaminidases in GM2-gangliosidosis.
pubmed:publicationType
Journal Article, Research Support, Non-U.S. Gov't