Journal of Lipid Research, Vol 23, 380-390, Copyright © 1982 by Lipid Research, Inc.

ARTICLES

The characterization of lipoproteins in the high density fraction obtained from patients with familial lecithin:cholesterol acyltransferase deficiency and their interaction with cultured human fibroblasts

AK Soutar, BL Knight and NB Myant

Lipoproteins of density 1.063--1.21 g/ml were isolated from the plasma of three sisters of Irish origin with familial LCAT deficiency. Fractionation of the lipoproteins on the basis of particle size by chromatography on Sephacryl S-300 permitted partial separation of two major and at least three other minor components which differed in their lipid:protein ratio and their apolipoprotein content. One of the major components was a small spherical lipoprotein whose sole apolipoprotein was apoA-I; the second major component contained predominantly apoA-I, together with apoE, and in addition, an apolipoprotein of molecular weight 46,000 that was not cleaved by reduction of disulfide bonds, and which was identified as apoA-IV. This apoprotein has not previously been detected in the lipoproteins of LCAT-deficient patients. A second apoE-containing lipoprotein, which contained apoA-I and apoE in a ratio of approximately 2:1, was also present as a minor component, together with two or more minor components whose apoproteins were comprised of apoA-I and apoC. The apoE-containing lipoproteins competed efficiently with 125I-labeled LDL for binding to high affinity LDL-receptor sites on the surface of cultured human skin fibroblasts. The ability to bind to the LDL-receptor was directly proportional to the apoE content of the lipoproteins, even when other apoproteins, with the exception of apoB, were present in relatively large proportions. ApoE-containing 125I-labeled lipoproteins from an LCAT-deficient subject were also taken up and degraded by the cultured cells.
CiteULike    Complore    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this?

This article has been cited by other articles:

				L. A. Cox, S. Birnbaum, M. C. Mahaney, D. L. Rainwater, J. T. Williams, and J. L. VandeBerg Identification of Promoter Variants in Baboon Endothelial Lipase That Regulate High-Density Lipoprotein Cholesterol Levels Circulation, September 4, 2007; 116(10): 1185 - 1195. [Abstract] [Full Text] [PDF]

				B. Zhang, P. Fan, E. Shimoji, H. Xu, K. Takeuchi, C. Bian, and K. Saku Inhibition of Cholesteryl Ester Transfer Protein Activity by JTT-705 Increases Apolipoprotein E-Containing High-Density Lipoprotein and Favorably Affects the Function and Enzyme Composition of High-Density Lipoprotein in Rabbits Arterioscler. Thromb. Vasc. Biol., October 1, 2004; 24(10): 1910 - 1915. [Abstract] [Full Text] [PDF]

				B. R. Scott, D. C. McManus, V. Franklin, A. G. McKenzie, T. Neville, D. L. Sparks, and Y. L. Marcel The N-terminal Globular Domain and the First Class A Amphipathic Helix of Apolipoprotein A-I Are Important for Lecithin:Cholesterol Acyltransferase Activation and the Maturation of High Density Lipoprotein in Vivo J. Biol. Chem., December 21, 2001; 276(52): 48716 - 48724. [Abstract] [Full Text] [PDF]