Plasma Lipoproteins in Familial Lecithin:Cholesterol Acyltransferase Deficiency: Effects of Dietary Manipulation

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Abstract
To study the metabolism of the abnormal plasma lipoproteins in familial lecithin:cholesterol acyltransferase deficiency we performed five dietary experiments designed to perturb their distribution and composition. Four patients with the disease were given successive diets that differed in triglyceride, carbohydrate, or cholesterol content, and after each dietary period the lipoproteins were analyzed by combinations of preparative and analytical ultracentrifuga-tion, gel filtration, chromatography, and disc gel electrophoresis. Lowering the intake of long chain, dietary trigylceride descreased 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 these effects, but increasing the intake of carbohydrate did not. Furthermore, decreasing the dietary cholesterol without lowering the dietary triglyceride only decreased the concentration of plasma cholesteryl ester. We conclude 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, two polar lipids that form a substantial part of the surfaces of chylomicrons, components of chylomicron surfaces may accumulate in the patients' plasma following enzymic removal of chylomicron triglyceride and contribute to several of the abnormal lipoproteins.

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