Familial Defective Apolipoprotein B-100: Low Density Lipoproteins with Abnormal Receptor Binding

• Published in: Proceedings of the National Academy of Sciences - PNAS Vol. 84; no. 19; pp. 6919 - 6923
• Main Authors: Innerarity, Thomas L., Weisgraber, Karl H., Arnold, Kay S., Mahley, Robert W., Krauss, Ronald M., Vega, Gloria L., Grundy, Scott M.
• Format: Journal Article
• Language: English
• Published: Washington, DC National Academy of Sciences of the United States of America 01.10.1987; National Acad Sciences
• Subjects: Analytical, structural and metabolic biochemistry; Apolipoprotein B-100; Apolipoproteins B - blood; Apolipoproteins B - genetics; Apolipoproteins B - metabolism; Biological and medical sciences; Brothers; Cells, Cultured; Cholesterols; Family members; Fibroblasts; Fibroblasts - metabolism; Fundamental and applied biological sciences. Psychology; Heterozygotes; Humans; Hypercholesterolemia; Hyperlipoproteinemia Type II - blood; Hyperlipoproteinemia Type II - genetics; Infant; Kinetics; Lipids; Lipoproteins; Lipoproteins, LDL - blood; Lipoproteins, myelin; Male; man; Proteins; Receptors; Receptors, LDL - metabolism; Sons; Human; Family study; Molecular interaction; Clearance; Apolipoproteins; Metabolism; Cholesterol; Membrane receptor; Hypercholesterolemia; Atherosclerosis; β-Lipoprotein; Ratio; Defectivity

Previous in vivo turnover studies suggested that retarded clearance of low density lipoproteins (LDL) from the plasma of some hypercholesterolemic patients is due to LDL with defective receptor binding. The present study examined this postulate directly by receptor binding experiments. The LDL from...