Plasma lipoproteins in familial lecithin: cholesterol acyltransferase deficiency: effects of dietary manipulation

Glomset, J.A.; Norum, K.R.; Nichols, A.V.; King, W.C.; Mitchell, C.D.; Applegate, K.R.; Gong, E.L.; Gjone, E.

Scandinavian Journal of Clinical and Laboratory Investigation. Supplementum 142: 3-30


ISSN/ISBN: 0085-591X
PMID: 169566
DOI: 10.1080/00365517509108157
Accession: 000461024

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To study the metabolism of the abnormal plasma lipoproteins in familial lecithin:cholesterol acyltransferase deficiency 5 dietary experiments were made to upset their distribution and composition. Four patients were given successive diets that differed in triglyceride, carbohydrate or cholesterol content, and after each dietary period the lipoproteins were analysed by combinations of preparative and analytical ultracentrifugation, gel filtration, chromatography and disc gel electrophoresis. Reducing the intake of long-chain dietary triglyceride decreased the concentrations of the large very-low-density lipoproteins, the large and intermediate low-density lipoproteins and the small high-density lipoproteins by as much as 79%, but either increased or did not change the concentrations of the small very-low- and low-density lipoproteins. Re-adding long-chain triglyceride to the diet generally reversed those effects, but increasing the intake of carbohydrate did not. Decreasing dietary cholesterol but not triglyceride decreased only the concentration of plasma cholesterol ester. It was concluded that the concentrations of the large very-low- and low-density lipoproteins, the intermediate-sized low-density lipoproteins and the small high-density lipoproteins are related to the absorption and subsequent transport of long-chain dietary fatty acids. Since these lipoproteins are rich in unesterified cholesterol and lecithin, components of chylomicron surfaces may accumulate in the plasma after enzymic removal of chylomicron triglyceride and contribute to several of the abnormal lipoproteins.