Literature DB >> 68276

Type-III Hyperlipoproteinaemia ("remnant removal disease"). Insight into the pathogenetic mechanism.

A Chait, J D Brunzell, J J Albers, W R Hazzard.   

Abstract

The metabolism of very-low-density lipoprotein (V.L.D.L.) was studied in a patient with type-III hyperlipoproteinaemia. After injection of radioiodinated V.L.D.L. from a donor with endogenous hypertriglyceridaemia (in a type-IV pattern), clearance of radioactivity from the apoprotein-B moiety of V.L.D.L. was slow and appearance in low-density lipoprotein (L.D.L.) was delayed, suggesting defective catabolism of V.L.D.L. in type-III hyperlipoproteinaemia. Despite a 60% increase in the rate of V.L.D.L. production, therapy with oestrogen (ethinyloestradiol 1 microng/kg/day) resulted in a decrease in plasma-lipid concentrations, normalisation of lipoprotein composition, and correction of the defect in V.L.D.L. catabolism with increased conversion to L.D.L. Thus, in type-III hyperlipoproteinaemia, the primary defect seems to be impaired catabolism of V.L.D.L., which is corrected with oestrogen therapy.

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Year:  1977        PMID: 68276     DOI: 10.1016/s0140-6736(77)92717-9

Source DB:  PubMed          Journal:  Lancet        ISSN: 0140-6736            Impact factor:   79.321


  16 in total

Review 1.  Plasma lipoproteins, lipid transport, and atherosclerosis: recent developments.

Authors:  N E Miller
Journal:  J Clin Pathol       Date:  1979-07       Impact factor: 3.411

Review 2.  Hypertriglyceridaemia in diabetes.

Authors:  W Stuart A Smellie
Journal:  BMJ       Date:  2006-12-16

3.  Pre-beta-very low density lipoproteins as precursors of beta-very low density lipoproteins. A model for the pathogenesis of familial dysbetalipoproteinemia (type III hyperlipoproteinemia).

Authors:  D A Chappell
Journal:  J Clin Invest       Date:  1988-08       Impact factor: 14.808

4.  Lipolytic surface remnants of triglyceride-rich lipoproteins are cytotoxic to macrophages but not in the presence of high density lipoprotein. A possible mechanism of atherogenesis?

Authors:  B H Chung; J P Segrest; K Smith; F M Griffin; C G Brouillette
Journal:  J Clin Invest       Date:  1989-04       Impact factor: 14.808

5.  Degradation of lipoproteins by human monocyte-derived macrophages. Evidence for two distinct processes for the degradation of abnormal very-low-density lipoprotein from subjects with type III hyperlipidaemia.

Authors:  A K Soutar; B L Knight
Journal:  Biochem J       Date:  1984-02-15       Impact factor: 3.857

6.  Metabolism of apolipoproteins B-48 and B-100 of triglyceride-rich lipoproteins in patients with familial dysbetalipoproteinemia.

Authors:  A F Stalenhoef; M J Malloy; J P Kane; R J Havel
Journal:  J Clin Invest       Date:  1986-09       Impact factor: 14.808

7.  Different patterns of postprandial lipoprotein metabolism in normal, type IIa, type III, and type IV hyperlipoproteinemic individuals. Effects of treatment with cholestyramine and gemfibrozil.

Authors:  M S Weintraub; S Eisenberg; J L Breslow
Journal:  J Clin Invest       Date:  1987-04       Impact factor: 14.808

8.  Sex differences in long chain fatty acid utilization and fatty acid binding protein concentration in rat liver.

Authors:  R K Ockner; D A Burnett; N Lysenko; J A Manning
Journal:  J Clin Invest       Date:  1979-07       Impact factor: 14.808

9.  Sex steroid modulation of fatty acid utilization and fatty acid binding protein concentration in rat liver.

Authors:  R K Ockner; N Lysenko; J A Manning; S E Monroe; D A Burnett
Journal:  J Clin Invest       Date:  1980-05       Impact factor: 14.808

Review 10.  Remnants of the Triglyceride-Rich Lipoproteins, Diabetes, and Cardiovascular Disease.

Authors:  Alan Chait; Henry N Ginsberg; Tomas Vaisar; Jay W Heinecke; Ira J Goldberg; Karin E Bornfeldt
Journal:  Diabetes       Date:  2020-04       Impact factor: 9.461

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