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rdf:type |
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lifeskim:mentions |
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pubmed:issue |
1
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pubmed:dateCreated |
1982-7-8
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pubmed:abstractText |
Skin fibroblast cultures from patients with I-cell disease (mucolipidosis II) are characterized by multiple lysosomal enzyme deficiencies The present studies deal with the consequences of these deficiencies with respect to the metabolism of plasma low-density lipoproteins. Degradation of the protein moiety was defective in I-cells compared with control cells, but the binding and internalization of low density lipoprotein were much less affected. Measurements of low-density lipoprotein degradation in homogenates demonstrated directly for the first time a deficiency of acid proteinase activity in I-cell fibroblasts. Comparison of results in 6-h incubations with those in 24-h incubations showed accumulation of intracellular low-density lipoprotein in I-cell fibroblasts and an accelerating rate of degradation, possibly attributable to intracellular accumulation of low-density lipoprotein substrate. The significance of these findings with respect to low-density lipoprotein metabolism in vivo is discussed.
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pubmed:grant |
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pubmed:commentsCorrections |
http://linkedlifedata.com/resource/pubmed/commentcorrection/7082306-1269176,
http://linkedlifedata.com/resource/pubmed/commentcorrection/7082306-13566175,
http://linkedlifedata.com/resource/pubmed/commentcorrection/7082306-170273,
http://linkedlifedata.com/resource/pubmed/commentcorrection/7082306-172501,
http://linkedlifedata.com/resource/pubmed/commentcorrection/7082306-174096,
http://linkedlifedata.com/resource/pubmed/commentcorrection/7082306-177050,
http://linkedlifedata.com/resource/pubmed/commentcorrection/7082306-194923,
http://linkedlifedata.com/resource/pubmed/commentcorrection/7082306-197883,
http://linkedlifedata.com/resource/pubmed/commentcorrection/7082306-35150,
http://linkedlifedata.com/resource/pubmed/commentcorrection/7082306-42439,
http://linkedlifedata.com/resource/pubmed/commentcorrection/7082306-4328711,
http://linkedlifedata.com/resource/pubmed/commentcorrection/7082306-4345092,
http://linkedlifedata.com/resource/pubmed/commentcorrection/7082306-4346288,
http://linkedlifedata.com/resource/pubmed/commentcorrection/7082306-4368448,
http://linkedlifedata.com/resource/pubmed/commentcorrection/7082306-526296,
http://linkedlifedata.com/resource/pubmed/commentcorrection/7082306-6262380,
http://linkedlifedata.com/resource/pubmed/commentcorrection/7082306-6452876,
http://linkedlifedata.com/resource/pubmed/commentcorrection/7082306-6989821,
http://linkedlifedata.com/resource/pubmed/commentcorrection/7082306-6989822,
http://linkedlifedata.com/resource/pubmed/commentcorrection/7082306-731127,
http://linkedlifedata.com/resource/pubmed/commentcorrection/7082306-7356669,
http://linkedlifedata.com/resource/pubmed/commentcorrection/7082306-7391040,
http://linkedlifedata.com/resource/pubmed/commentcorrection/7082306-7407073,
http://linkedlifedata.com/resource/pubmed/commentcorrection/7082306-822246
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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 |
Jan
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pubmed:issn |
0264-6021
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pubmed:author |
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pubmed:issnType |
Print
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pubmed:day |
15
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pubmed:volume |
202
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
183-90
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pubmed:dateRevised |
2010-9-13
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pubmed:meshHeading |
pubmed-meshheading:7082306-Cells, Cultured,
pubmed-meshheading:7082306-Fibroblasts,
pubmed-meshheading:7082306-Humans,
pubmed-meshheading:7082306-Hydroxymethylglutaryl CoA Reductases,
pubmed-meshheading:7082306-Hyperlipoproteinemia Type II,
pubmed-meshheading:7082306-Infant,
pubmed-meshheading:7082306-Ketocholesterols,
pubmed-meshheading:7082306-Lipoproteins, LDL,
pubmed-meshheading:7082306-Mucolipidoses,
pubmed-meshheading:7082306-Protein Binding,
pubmed-meshheading:7082306-Skin
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pubmed:year |
1982
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pubmed:articleTitle |
Defective catabolism of low-density lipoprotein by fibroblasts from patients with I-cell disease.
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pubmed:publicationType |
Journal Article,
Comparative Study,
Research Support, U.S. Gov't, P.H.S.,
Research Support, Non-U.S. Gov't
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