Idiopathic Hypercholesterolemia: Demonstration of an Impaired Feedback Control of Cholesterol Synthesis in vivo

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Abstract
The influence of dietary cholesterol on the synthesis of cholesterol by the liver was studied in liver biopsy samples from human infants. Dietary cholesterol in amounts of 3 to 4 g/day suppressed almost completely the synthesis of cholesterol by the liver. Acetate-C14 was orally administered to the infants fed normal or high-cholesterol diet, and the appearance of labeled lipids in the plasma was studied. The feeding of cholesterol depressed the incorporation of acetate-C14 into the plasma cholesterol fraction. In a patient with idiopathic hypercholesterolemia, the feeding of cholesterol had no inhibitory effect upon the incorporation of acetate-C14 into the plasma cholesterol fraction. The results of these studies indicate that infants do normally possess a feedback control of hepatic cholesterol synthesis and that this mechanism is impaired in an infant with idiopathic hypercholesterolemia.