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Predicate | Object |
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rdf:type | |
lifeskim:mentions | |
pubmed:issue |
2
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pubmed:dateCreated |
1984-5-24
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pubmed:abstractText |
We have prepared a new radiolabeled substrate (N-[3H]acetylglucosamine-glucuronic acid-N-[3H]acetylglucosamine), from hyaluronic acid, for an assay of beta-N-acetylhexosaminidase activity. Using this substrate, we found a striking deficiency of beta-N-acetylhexosaminidase activity in cultured skin fibroblasts and in liver homogenates from patients with Tay-Sachs disease. DEAE-cellulose chromatography at pH 6.0 revealed that both isoenzymes A and B of beta-N-acetylhexosaminidase from normal liver participated in the catabolism of hyaluronic acid. There were, however, major differences in substrate specificities between isoenzymes A and B. Our results indicate that this substrate should be useful for enzymatic diagnosis of Tay-Sachs disease.
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pubmed:language |
eng
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pubmed:journal | |
pubmed:citationSubset |
IM
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pubmed:chemical | |
pubmed:status |
MEDLINE
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pubmed:month |
Feb
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pubmed:issn |
0009-8981
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pubmed:author | |
pubmed:issnType |
Print
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pubmed:day |
28
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pubmed:volume |
137
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
159-68
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pubmed:dateRevised |
2007-11-15
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pubmed:meshHeading |
pubmed-meshheading:6231138-Cells, Cultured,
pubmed-meshheading:6231138-Chromatography, DEAE-Cellulose,
pubmed-meshheading:6231138-Clinical Enzyme Tests,
pubmed-meshheading:6231138-Fibroblasts,
pubmed-meshheading:6231138-Hexosaminidases,
pubmed-meshheading:6231138-Humans,
pubmed-meshheading:6231138-Isoenzymes,
pubmed-meshheading:6231138-Liver,
pubmed-meshheading:6231138-Substrate Specificity,
pubmed-meshheading:6231138-Tay-Sachs Disease,
pubmed-meshheading:6231138-Trisaccharides,
pubmed-meshheading:6231138-beta-N-Acetylhexosaminidases
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pubmed:year |
1984
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pubmed:articleTitle |
Diagnosis of Tay-Sachs disease by estimation of beta-N-acetylhexosaminidase activity using a radiolabeled hyaluronic acid-derived trisaccharide substrate.
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pubmed:publicationType |
Journal Article
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