Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
1
pubmed:dateCreated
1984-5-11
pubmed:abstractText
A fluorescent derivative of glucosyl ceramide was synthesized by covalently linking a fluorescent fatty acid, 12-[N-methyl-N-(7-nitrobenz-2-oxa-1,3-diazol-4-yl)] aminododecanoic acid to the amino group of sphingosyl-1-O-beta-D-glucoside, glucosyl sphingosine. For hydrolysis by glucocerebrosidase, this substrate was dispersed in mixed micelles with Triton X-100 and sodium taurocholate or in unilamellar liposomes with phosphatidylcholine and the negatively charged lipid, dicetylphosphate. In either micellar or liposomal dispersions of the fluorescent substrate, reaction rates were linear with time and protein concentration, and saturation kinetics were observed. The rate of hydrolysis of this fluorescent substrate was equal to that obtained with radiolabeled glucosyl ceramide. The fluorescent glucosyl ceramide was used to determine glucocerebrosidase activity in extracts of human leukocytes, cultured skin fibroblasts, and various tissues as well as in partially purified splenic and placental glucocerebrosidase preparations. This fluorescent derivative of the natural substrate was not hydrolyzed by aryl beta-glucosidase(s), thereby facilitating the specific and reliable diagnosis of heterozygotes and homozygotes with Gaucher disease.
pubmed:grant
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Jan
pubmed:issn
0003-2697
pubmed:author
pubmed:issnType
Print
pubmed:volume
136
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
223-34
pubmed:dateRevised
2008-11-21
pubmed:meshHeading
pubmed:year
1984
pubmed:articleTitle
Synthesis of a fluorescent derivative of glucosyl ceramide for the sensitive determination of glucocerebrosidase activity.
pubmed:publicationType
Journal Article, Research Support, U.S. Gov't, P.H.S., Research Support, Non-U.S. Gov't