Cholesteryl Ester Accumulation in Mouse Peritoneal Macrophages Induced by f-Migrating Very Low Density Lipoproteins from Patients with Atypical Dysbetalipoproteinemia

tients in a kindred with atypical dysbetalipopro-teinemia induced marked cholesteryl ester accumu-lation in mouse peritoneal macrophages. The affected family members had severe hypercholesterolemia and hypertriglyceridemia, xanthomatosis, premature vas-cular disease, the apo-E3/3 phenotype, and a predom-inance of cholesterol-rich f3-very low density lipopro-teins (f,-VLDL) in the d < 1.006 fraction. When in-cubated with mouse peritoneal macrophages, the d < 1.006 lipoproteins or,B-VLDL from the affected family members stimulated cholesteryl ['4C]oleate syn-thesis 15- to 30-fold above that caused by normal, con-trol d < 1.006 lipoproteins (VLDL). The ability of the #l-VLDL to stimulate macrophage cholesteryl ester accumulation was greatly reduced as a consequenc