Dysbetalipoproteinaemia : clinical and laboratory aspects, changes in lipoprotein composition and remnant metabolism associated with lipid lowering drugs

Abstract

This thesis examines and reviews multiple aspects of dysbetalipoproteinaemia. Dysbetalipoproteinaemia is a severe, highly atherogenic mixed hyperlipidaernia characterized by the accumulation of remnants of triglyceride-rich lipoproteins. Genetic susceptibility is conferred by mutations in apolipoproteinE, but manifest hyperlipidaemia often only occurs in the presence of metabolic abnormalities that influence lipoprotein production or clearance. Normal lipid metabolism is briefly reviewed to enable a better understanding of the pathophysiology of dysbetalipoproteinaernia. Hepatic remnant clearance is reviewed in detail with particular emphasis on the roles of all hepatic lipoprotein receptors. The importance of differential hepatic lipoprotein receptor binding affinities according to apoE mutation is emphasized.