Linked Life Data

3126354

Source:http://linkedlifedata.com/resource/pubmed/id/3126354

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
4
pubmed:dateCreated
1988-4-12
pubmed:abstractText
Cholesterol synthesis, esterification and efflux were investigated in cultured fibroblasts from patients with Niemann-Pick disease type C. Sterol synthesis from sodium acetate was markedly increased in the two Niemann-Pick disease type C strains as compared to controls, either in the presence or absence of exogenous cholesterol supply by low-density lipoproteins. By contrast, cholesterol esterification was about 2-3-fold reduced when measured by oleic acid incorporation into cholesteryl esters and 10-15-fold reduced when measured with labelled free cholesterol as precursor, although acylcoenzyme-A:cholesterol acyltransferase activity was normal when studied in vitro on cell homogenates. Chase experiments with 14C-cholesterol demonstrated that the rate of cholesterol efflux was decreased by about 3-4-fold in fibroblasts from patients with Niemann-Pick disease type C. These results provide further evidence for alterations of sterol metabolism in Niemann-Pick disease type C and support the hypothesis of a trapping of exogenous cholesterol, which cannot enter the regulatory intracellular pools.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:issn
0141-8955
pubmed:author
pubmed:issnType
Print
pubmed:volume
10
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
339-46
pubmed:dateRevised
2007-3-21
pubmed:meshHeading
pubmed:year
1987
pubmed:articleTitle
Alterations in cholesterol metabolism in cultured fibroblasts from patients with Niemann-Pick disease type C.
pubmed:affiliation
Laboratoire de Biochimie, CNRS UA 524, Faculté de Médecine Saint-Antoine, Paris, France.
pubmed:publicationType
Journal Article