Neutral lipids of plasma and adipose tissue in a-betalipo-proteinaemia

Abstract

Plasma triglyceride concentrations were found to be very low in 4 children with a-betalipoproteinaemia; studies in one child after a meal containing 2 g/kg of corn oil showed no rise in plasma triglyceride in the post-absorptive state. However, evidence for absorption of the corn oil was provided by the finding of a rise in the proportion of linoleic acid in the plasma non-esterified fatty acid (NEFA) fraction during the first 6 h, and in cholesterol ester after 24 h. The fatty acid composition of adipose tissue triglyceride showed an increased proportion of palmitoleic acid, indicative of lipogenesis, and reduced linoleic acid, a consequence of impaired absorption; reduction in stearic acid was also found. The fatty acid pattern of plasma NEFA in general reflected that of the adipose tissue though in plasma NEFA normal levels of linoleic acid were found in the two patients receiving supplementary corn oil. In all children linoleic acid in cholesterol ester was very low. Plasma post-heparin lipolytic activity was grossly reduced in the three children in whom it was estimated.