Fat Transport in Abetalipoproteinemia

Abstract

Over a four-day period an 11-year-old boy with abetalipoproteinemia was given intravenously 2150 ml of citrated plasma from a subject with hyperbetalipoproteinemia. The recipient's plasma β-protein cholesterol concentration was raised from zero to a maximum of 285 mg per deciliter. Despite high plasma levels of β-lipoprotein for more than six days, plasma glycerides never rose above 22 mg per deciliter, and no evidence was found for the formation of chylomicrons or pre-β-lipoproteins. The infused β-lipoprotein disappeared from the recipient's plasma with a half-time of 2.3 days.