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pubmed-article:6434321pubmed:abstractTextIn the rare familial disorder fish-eye disease, hypertriglyceridaemia is associated with elevated levels of very-low-density lipoprotein (VLDL) and enrichment of low-density lipoprotein (LDL) with triglyceride. The kinetic basis of the dyslipoproteinaemia was investigated by studying the metabolism of the apolipoprotein-B moeity of VLDL, intermediate-density lipoprotein (IDL) and LDL in a 68-year-old woman with this condition. The major kinetic abnormality was a pronounced reduction in the rate of fractional conversion of VLDL-B to IDL-B and of IDL-B to LDL-B, suggesting that the dyslipoproteinaemia represents accumulation in plasma of partly degraded products of VLDL metabolism. This kinetic disorder has features in common with type-III hyperlipoproteinaemia. In studies in vitro no defect in the enzyme, activator or substrate components of the lipoprotein lipase or hepatic lipase systems was observed.lld:pubmed
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pubmed-article:6434321pubmed:dateRevised2006-11-15lld:pubmed
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pubmed-article:6434321pubmed:articleTitleStudies of lipoprotein metabolism in a patient with fish-eye disease.lld:pubmed
pubmed-article:6434321pubmed:publicationTypeJournal Articlelld:pubmed
pubmed-article:6434321pubmed:publicationTypeResearch Support, Non-U.S. Gov'tlld:pubmed
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