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

John A. Glomset Dept. of Medicine and Regional Primate Research Center, University of Washington, Seattle, Washington, USA; Institute for Nutrition Research and Medical Department A, School of Medicine, University of Oslo, Norway; Donner Research Laboratory, University of California, Berkeley, California, USA

Kaare R. Norum Dept. of Medicine and Regional Primate Research Center, University of Washington, Seattle, Washington, USA; Institute for Nutrition Research and Medical Department A, School of Medicine, University of Oslo, Norway; Donner Research Laboratory, University of California, Berkeley, California, USA

Alex V. Nichols Dept. of Medicine and Regional Primate Research Center, University of Washington, Seattle, Washington, USA; Institute for Nutrition Research and Medical Department A, School of Medicine, University of Oslo, Norway; Donner Research Laboratory, University of California, Berkeley, California, USA

Weiling C. King Dept. of Medicine and Regional Primate Research Center, University of Washington, Seattle, Washington, USA; Institute for Nutrition Research and Medical Department A, School of Medicine, University of Oslo, Norway; Donner Research Laboratory, University of California, Berkeley, California, USA

Carolyn D. Mitchell Dept. of Medicine and Regional Primate Research Center, University of Washington, Seattle, Washington, USA; Institute for Nutrition Research and Medical Department A, School of Medicine, University of Oslo, Norway; Donner Research Laboratory, University of California, Berkeley, California, USA

Kenneth R. Applegate Dept. of Medicine and Regional Primate Research Center, University of Washington, Seattle, Washington, USA; Institute for Nutrition Research and Medical Department A, School of Medicine, University of Oslo, Norway; Donner Research Laboratory, University of California, Berkeley, California, USA

Elaine L. Gong Dept. of Medicine and Regional Primate Research Center, University of Washington, Seattle, Washington, USA; Institute for Nutrition Research and Medical Department A, School of Medicine, University of Oslo, Norway; Donner Research Laboratory, University of California, Berkeley, California, USA

Egil Gjone Dept. of Medicine and Regional Primate Research Center, University of Washington, Seattle, Washington, USA; Institute for Nutrition Research and Medical Department A, School of Medicine, University of Oslo, Norway; Donner Research Laboratory, University of California, Berkeley, California, USA

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

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

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