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rdf:type |
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lifeskim:mentions |
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pubmed:issue |
3
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pubmed:dateCreated |
1996-1-18
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pubmed:abstractText |
We have adapted two methods to evaluate the beta-hexosaminidase (HEX) enzymatic activity in cultured cells, based on the use of (i) the fluorogenic substrate 4-methylumbelliferyl-6-sulfo-2-acetamido-2- deoxy-beta-D-glucopyranoside (MU-GlcNAc-6-SO4) and (ii) the naphthol AS-BI-N-acetyl-beta-D-glucosaminide and hexazotized pararosaniline. We demonstrate that both methods could be used for the HEX isoenzymes by comparing wild-type and mutant human fibroblast cell lines, deficient for either an alpha or beta subunit from Tay-Sachs and Sandhoff patients. This in situ cytochemical assessment of HEX activity offers a rapid evaluation to study the expression of this enzyme in a heterogeneous cell population such as in gene transfer experiments.
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pubmed:language |
eng
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pubmed:journal |
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pubmed:citationSubset |
IM
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pubmed:chemical |
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pubmed:status |
MEDLINE
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pubmed:month |
Sep
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pubmed:issn |
0736-6205
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pubmed:author |
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pubmed:issnType |
Print
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pubmed:volume |
19
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
434-40
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pubmed:dateRevised |
2007-11-15
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pubmed:meshHeading |
pubmed-meshheading:7495557-Acetylglucosamine,
pubmed-meshheading:7495557-Cells, Cultured,
pubmed-meshheading:7495557-Fibroblasts,
pubmed-meshheading:7495557-Glucuronates,
pubmed-meshheading:7495557-Histocytochemistry,
pubmed-meshheading:7495557-Humans,
pubmed-meshheading:7495557-Hymecromone,
pubmed-meshheading:7495557-Isoenzymes,
pubmed-meshheading:7495557-Mutation,
pubmed-meshheading:7495557-Naphthols,
pubmed-meshheading:7495557-Sandhoff Disease,
pubmed-meshheading:7495557-Spectrometry, Fluorescence,
pubmed-meshheading:7495557-Tay-Sachs Disease,
pubmed-meshheading:7495557-beta-N-Acetylhexosaminidases
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pubmed:year |
1995
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pubmed:articleTitle |
In situ assessment of beta-hexosaminidase activity.
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pubmed:affiliation |
Genetics and Molecular Immunology Section, NEI, National Institutes of Health, Bethesda, MD 20892-1858, USA.
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pubmed:publicationType |
Technical Report
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