Pathogenesis of type III hyperlipoproteinemia (dysbetalipoproteinemia): questions, quandaries, and paradoxes

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Pathogenesis of type III hyperlipoproteinemia (dysbetalipoproteinemia). Questions, quandaries, and paradoxes.

Type III hyperlipoproteinemia (HLP) is a genetic disorder characterized by accumulation of remnant lipoproteins in the plasma and development of premature atherosclerosis. Although receptor binding-defective forms of apolipoprotein (apo) E are the common denominator in this disorder, a number of apparent paradoxes concerning its pathogenesis still exist. However, studies in transgenic animals a...

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High receptor binding affinity of lipoproteins in atypical dysbetalipoproteinemia (type III hyperlipoproteinemia).

Familial dysbetalipoproteinemia (or type III hyperlipoproteinemia) is characterized by the presence of abnormal, cholesteryl ester-rich beta-very low density lipoproteins (beta-VLDL) in the plasma. Subjects with typical dysbetalipoproteinemia are homozygous for an amino acid substitution in apolipoprotein (apo-) E at residue 158 and have defective apo-E-mediated binding of both pre-beta-VLDL an...

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Pathogenesis of hyperlipoproteinemia.

In the past 25 years, great advances have been made in our understanding of lipoprotein metabolism and of the causation of hyperlipidemia. These advances have been due in part to the study of patients with unusual defects in lipoprotein metabolism. Follow-up investigations into the specific metabolic defects in these patients have revealed qualitative abnormalities in the structure and function...

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ژورنال

عنوان ژورنال: Journal of Lipid Research

سال: 1999

ISSN: 0022-2275

DOI: 10.1016/s0022-2275(20)32417-2