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Predicate | Object |
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rdf:type | |
lifeskim:mentions | |
pubmed:issue |
4
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pubmed:dateCreated |
1992-11-12
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pubmed:abstractText |
Cell lines derived from the sphingomyelinosis (gene symbol, spm) mouse were established from homozygous (spm/spm) and heterozygous (spm/+) embryos according to a rigid 3T3 transfer schedule. The SPM-3T3 cells derived from a homozygous embryo showed extensive accumulation of intracellular cholesterol, attenuated esterification of exogenously added cholesterol and increased de novo cholesterol synthesis, when compared to SPMH-3T3 cells derived from a heterozygous embryo. The phenotypic abnormalities were very similar to those observed in fibroblasts from patients with Niemann-Pick disease type C (NP-C), in which a defect in the intracellular transport of unesterified cholesterol is suggested. The genetic defect in SPM-3T3 cells should be closely related to that in NP-C. The SPM-3T3 cell line is useful for biochemical and genetic studies on the regulation of intracellular cholesterol metabolism.
|
pubmed:language |
eng
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pubmed:journal | |
pubmed:citationSubset |
IM
|
pubmed:chemical | |
pubmed:status |
MEDLINE
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pubmed:month |
Aug
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pubmed:issn |
0386-7196
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pubmed:author | |
pubmed:issnType |
Print
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pubmed:volume |
17
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
|
pubmed:pagination |
229-35
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pubmed:dateRevised |
2006-11-15
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pubmed:meshHeading |
pubmed-meshheading:1394466-3T3 Cells,
pubmed-meshheading:1394466-Animals,
pubmed-meshheading:1394466-Biological Transport,
pubmed-meshheading:1394466-Cholesterol,
pubmed-meshheading:1394466-Disease Models, Animal,
pubmed-meshheading:1394466-Filipin,
pubmed-meshheading:1394466-Mice,
pubmed-meshheading:1394466-Mice, Neurologic Mutants,
pubmed-meshheading:1394466-Niemann-Pick Diseases
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pubmed:year |
1992
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pubmed:articleTitle |
A cell line derived from sphingomyelinosis mouse shows alterations in intracellular cholesterol metabolism similar to those in type C Niemann-Pick disease.
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pubmed:affiliation |
Division of Child Neurology, Institute of Neurological Sciences, Tottori University School of Medicine, Yonago, Japan.
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pubmed:publicationType |
Journal Article,
Research Support, Non-U.S. Gov't
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