| pubmed-article:7082306 | rdf:type | pubmed:Citation | lld:pubmed |
| pubmed-article:7082306 | lifeskim:mentions | umls-concept:C0030705 | lld:lifeskim |
| pubmed-article:7082306 | lifeskim:mentions | umls-concept:C0016030 | lld:lifeskim |
| pubmed-article:7082306 | lifeskim:mentions | umls-concept:C0023820 | lld:lifeskim |
| pubmed-article:7082306 | lifeskim:mentions | umls-concept:C0020725 | lld:lifeskim |
| pubmed-article:7082306 | lifeskim:mentions | umls-concept:C0699900 | lld:lifeskim |
| pubmed-article:7082306 | pubmed:issue | 1 | lld:pubmed |
| pubmed-article:7082306 | pubmed:dateCreated | 1982-7-8 | lld:pubmed |
| pubmed-article:7082306 | 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. | lld:pubmed |
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| pubmed-article:7082306 | pubmed:language | eng | lld:pubmed |
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| pubmed-article:7082306 | pubmed:citationSubset | IM | lld:pubmed |
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| pubmed-article:7082306 | pubmed:status | MEDLINE | lld:pubmed |
| pubmed-article:7082306 | pubmed:month | Jan | lld:pubmed |
| pubmed-article:7082306 | pubmed:issn | 0264-6021 | lld:pubmed |
| pubmed-article:7082306 | pubmed:author | pubmed-author:SteinbergDD | lld:pubmed |
| pubmed-article:7082306 | pubmed:author | pubmed-author:MillerA LAL | lld:pubmed |
| pubmed-article:7082306 | pubmed:author | pubmed-author:WilliamsJ CJC | lld:pubmed |
| pubmed-article:7082306 | pubmed:author | pubmed-author:WeinsteinD... | lld:pubmed |
| pubmed-article:7082306 | pubmed:issnType | Print | lld:pubmed |
| pubmed-article:7082306 | pubmed:day | 15 | lld:pubmed |
| pubmed-article:7082306 | pubmed:volume | 202 | lld:pubmed |
| pubmed-article:7082306 | pubmed:owner | NLM | lld:pubmed |
| pubmed-article:7082306 | pubmed:authorsComplete | Y | lld:pubmed |
| pubmed-article:7082306 | pubmed:pagination | 183-90 | lld:pubmed |
| pubmed-article:7082306 | pubmed:dateRevised | 2010-9-13 | lld:pubmed |
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| pubmed-article:7082306 | pubmed:meshHeading | pubmed-meshheading:7082306-... | lld:pubmed |
| pubmed-article:7082306 | pubmed:year | 1982 | lld:pubmed |
| pubmed-article:7082306 | pubmed:articleTitle | Defective catabolism of low-density lipoprotein by fibroblasts from patients with I-cell disease. | lld:pubmed |
| pubmed-article:7082306 | pubmed:publicationType | Journal Article | lld:pubmed |
| pubmed-article:7082306 | pubmed:publicationType | Comparative Study | lld:pubmed |
| pubmed-article:7082306 | pubmed:publicationType | Research Support, U.S. Gov't, P.H.S. | lld:pubmed |
| pubmed-article:7082306 | pubmed:publicationType | Research Support, Non-U.S. Gov't | lld:pubmed |
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