Mutations affecting the binding, internalization, and lysosomal hydrolysis of low density lipoprotein in cultured human fibroblasts, lymphocytes, and aortic smooth muscle cells.

Abstract

Studies comparing the metabolism of low density lipoprotein (LDL) in normal cells and in cells cultured from patients with homozygous familial hypercholesterolemia have disclosed the existence of a receptor for plasma LDL. This receptor has been identified on the surface of human fibroblasts, lymphocytes, and aortic smooth muscle cells. An extension of these studies to cell strains derived from patients with other single gene defects in cholesterol metabolism has provided additional insight into the normal mechanisms by which cells regulate their cholesterol content and how alterations in these genetic control mechanisms may predispose to atherosclerosis in man.

• Authors: M S Brown, R G Anderson, J L Goldstein
• Journal: Journal of supramolecular structure (J Supramol Struct) Vol. 6 Issue 1 Pg. 85-94 ( 1977) ISSN: 0091-7419 [Print] United States
• PMID: 197319 (Publication Type: Journal Article, Research Support, U.S. Gov't, P.H.S.)

Chemical References

• Cholesterol Esters
• Lipoproteins, HDL
• Lipoproteins, LDL
• Oleic Acids
• Receptors, Drug

Topics

• Aorta
• Cells, Cultured
• Cholesterol Esters (biosynthesis)
• Fibroblasts (metabolism)
• Humans
• Hypercholesterolemia (metabolism)
• Lipoproteins, HDL (metabolism)
• Lipoproteins, LDL (metabolism)
• Lymphocytes (metabolism)
• Lysosomes (metabolism)
• Muscle, Smooth (metabolism, ultrastructure)
• Mutation
• Oleic Acids (metabolism)
• Receptors, Drug (analysis)