JCI - Citations to Characterization of hepatic low density lipoprotein binding and cholesterol metabolism in normal and homozygous familial hypercholesterolemic subjects.

Citations to this article

Characterization of hepatic low density lipoprotein binding and cholesterol metabolism in normal and homozygous familial hypercholesterolemic subjects.

Published February 1, 1984
Citation Information: J Clin Invest. 1984;73(2):429-436. https://doi.org/10.1172/JCI111229.

View: Text | PDF

Research Article

Characterization of hepatic low density lipoprotein binding and cholesterol metabolism in normal and homozygous familial hypercholesterolemic subjects.

Text
PDF

Abstract

Patients with familial hypercholesterolemia have elevated levels of plasma low density lipoproteins (LDL), increased hepatic synthesis of apolipoprotein B-containing lipoproteins, defective binding of low density lipoproteins to fibroblasts, and premature atherosclerosis. The role of a hepatic low density lipoprotein receptor in normal man and its importance in the pathogenesis of familial hypercholesterolemia have not been previously determined. In the present study, direct comparison was made of the binding of LDL to hepatic membranes from normal and receptor-negative homozygous familial hypercholesterolemic subjects. The effects of calcium, EDTA, and temperature on the binding of lipoproteins to the hepatic membranes were also evaluated. At 4 degrees C, no significant difference in specific binding of LDL to hepatic membranes from normal and familial hypercholesterolemic subjects was observed. At 37 degrees C, both total and specific binding of LDL were significantly reduced in patients with familial hypercholesterolemia. Hepatic membrane binding of LDL from the two patients homozygous for receptor-negative familial hypercholesterolemia was 53 and 59% of normal. The activity of the rate-limiting enzyme in cholesterol biosynthesis, 3-hydroxy-3-methylglutaryl coenzyme A reductase was normal; however, the total hepatic cholesterol and cholesteryl ester content was significantly increased from 53 to 129%. These results indicate that patients with familial hypercholesterolemia have a defect in the interaction of hepatic membranes with low density lipoproteins. This defect may lead to accelerated atherosclerosis by decreasing the cellular catabolism of LDL and enhancing the production of LDL, which is characteristic of patients homozygous for familial hypercholesterolemia.

Authors

J M Hoeg, S J Demosky Jr, E J Schaefer, T E Starzl, H B Brewer Jr

Total citations by year

Year:	2022	2021	2012	2007	2005	2004	2002	2000	1997	1995	1994	1993	1992	1991	1990	1989	1988	1987	1986	1985	1984	Total
Citations:	1	1	1	1	1	1	3	1	1	2	1	1	3	6	4	2	4	4	6	7	2	53

Citation information

Citations to this article (53)

Title and authors	Publication	Year
Hepatocyte cholesterol content modulates glucagon receptor signalling McGlone ER, Ansell TB, Dunsterville C, Song W, Carling D, Tomas A, Bloom SR, Sansom MS, Tan T, Jones B	Molecular Metabolism	2022
Prevalence of corneal arcus and associated factors in a German population—Results from the Gutenberg Health Study J Wasielica-Poslednik, U Hampel, L Ries, R Faysal, A Schulz, JH Prochaska, PS Wild, I Schmidtmann, T Münzel, ME Beutel, KJ Lackner, N Pfeiffer, AK Schuster, L Calabresi	PloS one	2021
Methods in Enzymology JD Stone, AS Chervin, DH Aggen, DM Kranz	Protein Engineering for Therapeutics Part B	2012
Corneal arcus as coronary artery disease risk factor A Fernández, A Sorokin, PD Thompson	Atherosclerosis	2007
Fetuin-A, a hepatocyte-specific protein that binds Plasmodium berghei thrombospondin-related adhesive protein: a potential role in infectivity D Jethwaney, T Lepore, S Hassan, K Mello, R Rangarajan, W Jahnen-Dechent, D Wirth, AA Sultan	Infection and immunity	2005
Evidence of QTLs on chromosomes 1q42 and 8q24 for LDL-cholesterol and apoB levels in the HERITAGE Family Study MF Feitosa, IB Borecki, T Rankinen, T Rice, JP Després, YC Chagnon, J Gagnon, AS Leon, JS Skinner, C Bouchard, MA Province, DC Rao	Journal of lipid research	2004
Cholesterol lowering effects of a choleretic phloracetophenone in hypercholesterolemic hamsters P Piyachaturawat, P Srivoraphan, A Chuncharunee, P Komaratat, A Suksamrarn	European Journal of Pharmacology	2002
Modification of Serum Lipid Concentrations in Young Men Exposed to a Hot Environment H YAMAMOTO, K ZHENG, M ARIIZUMI	SANGYO EISEIGAKU ZASSHI	2002
Lipoproteins, nutrition, and heart disease EJ Schaefer	The American journal of clinical nutrition	2002
Governance of the concentration of plasma LDL: a reevaluation of the LDL receptor paradigm A D Sniderman, X J Zhang, K Cianflone	Atherosclerosis	2000
Identification of human liver carboxylesterase as one of the proteins involved in Plasmodium falciparum malaria sporozoite invasion in primary cultures of human hepatocytes JF van Pelt, HJ Moshage, E Depla, JP Verhave, SH Yap	Journal of Hepatology	1997
Dominant expression of type III hyperlipoproteinemia. Pathophysiological insights derived from the structural and kinetic characteristics of ApoE-1 (Lys146-->Glu) WA Mann, P Lohse, RE Gregg, R Ronan, JM Hoeg, LA Zech, HB Brewer	Journal of Clinical Investigation	1995
Lipids and atherosclerosis MJ Halpern	Molecular Aspects of Medicine	1995
ApoB metabolism in familial hypercholesterolemia. Inconsistencies with the LDL receptor paradigm WR Fisher, LA Zech, PW Stacpoole	Arteriosclerosis Thrombosis and Vascular Biology	1994
The Gordon Wilson Lecture. Plasma cholesterol: atherogenesis and mortality WR Fisher	Transactions of the American Clinical and Climatological Association	1993
Regulation of LDL Receptor, apoB, and apoE Protein and mRNA in Hep G2 Cells HG Kraft, SJ Demosky, K Schumacher, HB Brewer, JM Hoeg	DNA and Cell Biology	1992
Characterization of the low density lipoprotein receptor activity in buffalo rat liver (BRL-3A) cells M Santillo, P Mondola, F Santangelo, A Belfiore, I Tedesco, C Laezza, R Cerbone, A Romano, M Bifulco	Comparative Biochemistry and Physiology Part B: Comparative Biochemistry	1992
Free fatty acids activate a high-affinity saturable pathway for degradation of low-density lipoproteins in fibroblasts from a subject homozygous for familial hypercholesterolemia BE Bihain, FT Yen	Biochemistry	1992
Visualization of the interaction of native and modified lipoproteins with parenchymal, endothelial and Kupffer cells from human liver MF Kleinherenbrink-Stins, JH van de Boom, D Schouten, PJ Roholl, MN van der Heyde, A Brouwer, TJ Berkel, DL Knook	Hepatology	1991
Pharmacologic and Surgical Treatment of Dyslipidemic Children and Adolescents JM Hoeg	Annals of the New York Academy of Sciences	1991
Ursodeoxycholic acid increases low-density lipoprotein binding, uptake and degradation in isolated hamster hepatocytes B Bouscarel, H Fromm, S Ceryak, MM Cassidy	Biochemical Journal	1991
Uptake and degradation of human low-density lipoprotein by human liver parenchymal and Kupffer cells in culture JA Kamps, JK Kruijt, J Kuiper, TJ van Berkel	Biochemical Journal	1991
Complete down-regulation of low-density lipoprotein receptor activity in human liver parenchymal cells by β-very-low-density lipoprotein JA Kamps, J Kuiper, JK Kruijt, TJ van Berkel	FEBS Letters	1991
Identification of plasma membrane proteins involved in the hepatocyte invasion of Plasmodium falciparum sporozoites JF van Pelt, J Kleuskens, MR Hollingdale, JP Verhave, T Ponnudurai, JH Meuwissen, SH Yap	Molecular and Biochemical Parasitology	1991
Low density lipoprotein receptor-binding activity in human tissues: quantitative importance of hepatic receptors and evidence for regulation of their expression in vivo MJ Rudling, E Reihnér, K Einarsson, S Ewerth, B Angelin	Proceedings of the National Academy of Sciences	1990
Aspects génétiques et physiopathologiques des dyslipoprotéinémies athérogènes G Turpin, E Bruckert	La Revue de Médecine Interne	1990
Hormonal determinants of apolipoprotein B,E receptor expression in human liver. Positive association of receptor expression with plasma estrone concentration in middle-aged/elderly women MN Nanjee, DR Koritnik, J Thomas, NE Miller	Biochimica et Biophysica Acta (BBA) - Lipids and Lipid Metabolism	1990
Cholesterol Metabolism, Ldl, and the Ldl Receptor NB Myant	Cholesterol Metabolism, Ldl, and the Ldl Receptor	1990
Human hepatic low-density lipoprotein receptors: associations of receptor activities in vitro with plasma lipid and apolipoprotein concentrations in vivo MN Nanjee, NE Miller	Biochimica et Biophysica Acta (BBA) - Lipids and Lipid Metabolism	1989
Low-density-lipoprotein receptors in different rabbit liver cells MS Nenseter, O Myklebost, R Blomhoff, CA Drevon, A Nilsson, KR Norum, T Berg	Biochemical Journal	1989
Biochemical and pathological features of a modified strain of Watanabe heritable hyperlipidaemic rabbits PJ Gallagher, MN Nanjee, T Richards, WR Roche, NE Miller	Atherosclerosis	1988
Some metabolic characteristics of low-density lipoprotein subfractions, LDL-1 and LDL-2: in vitro and in vivo studies DW Swinkels, PN Demacker, HL Hak-Lemmers, MJ Mol, SH Yap, A Laar	Biochimica et Biophysica Acta (BBA) - Lipids and Lipid Metabolism	1988
Enhanced apolipoprotein E production with normal hepatic mRNA levels in the Watanabe heritable hyperlipidemic rabbit CJ Rall, JM Hoeg, RE Gregg, SW Law, JC Monge, MS Meng, LA Zech, HB Brewer	Arteriosclerosis (Dallas, Tex.)	1988
Role of low density lipoprotein receptor-dependent and -independent sites in binding and uptake of chylomicron remnants in rat liver Y Nagata, J Chen, AD Cooper	The Journal of biological chemistry	1988
Lipoprotein binding to cultured human hepatoma cells F Krempler, GM Kostner, W Friedl, B Paulweber, H Bauer, F Sandhofer	Journal of Clinical Investigation	1987
Immunoblotting and ligand blotting of the low-density lipoprotein receptor of human liver, HepG2 cells and HeLa cells JR Havinga, P Lohse, U Beisiegel	FEBS Letters	1987
Binding of low-density lipoprotein to monolayer cultures of rat hepatocytes is increased by insulin and decreased by dexamethasone AM Salter, SC Fisher, DN Brindley	FEBS Letters	1987
Estrogens induce low-density lipoprotein receptor activity and decrease intracellular cholesterol in human hepatoma cell line Hep G2 CF Semenkovich, RE Ostlund	Biochemistry	1987
A study of the structural heterogeneity of low-density lipoproteins in two patients homozygous for familial hypercholesterolaemia, one of phenotype E2/2 G Luc, MJ Chapman, JL Gennes, G Turpin	European Journal of Clinical Investigation	1986
Metabolism of low-density lipoproteins by cultured hepatocytes from normal and homozygous familial hypercholesterolemic subjects JM Hoeg, SB Edge, SJ Demosky, TE Starzl, T Triche, RE Gregg, HB Brewer	Biochimica et Biophysica Acta	1986
Characterization of the binding of human low-density lipoprotein to primary monolayer cultures of rat hepatocytes AM Salter, J Saxton, DN Brindley	Biochemical Journal	1986
The expressed human hepatic receptor for low-density lipoproteins differs from the fibroblast low-density lipoprotein receptor JM Hoeg, SJ Demosky, KJ Lackner, JC Osborne, C Oliver, HB Brewer	Biochimica et Biophysica Acta (BBA) - Lipids and Lipid Metabolism	1986
Impaired hepatocyte binding, uptake and degradation of glucosylated low-density lipoproteins JE Triau, J Arbetter, EJ Schaefer	Biochimica et Biophysica Acta (BBA) - Lipids and Lipid Metabolism	1986
Cultured human hepatocytes. Evidence for metabolism of low density lipoproteins by a pathway independent of the classical low density lipoprotein receptor SB Edge, JM Hoeg, T Triche, PD Schneider, HB Brewer	The Journal of biological chemistry	1986
Hepatic binding of triglyceride-rich lipoproteins in humans CH Florén	Scandinavian Journal of Clinical & Laboratory Investigation	1985
Apolipoprotein B synthesis in humans: Liver synthesizes only apolipoprotein B-100 SB Edge, JM Hoeg, PD Schneider, HB Brewer	Metabolism	1985
The effect of portacaval shunt on hepatic lipoprotein metabolism in familial hypercholesterolemia JM Hoeg, SJ Demosky, EJ Schaefer, TE Starzl, KA Porter, HB Brewer	Journal of Surgical Research	1985
Effects of combination cholestyramine-neomycin treatment on plasma lipoprotein concentrations in type II hyperlipoproteinemia JM Hoeg, MB Maher, KR Bailey, LA Zech, RE Gregg, DL Sprecher, HB Brewer	The American Journal of Cardiology	1985
Characterization of a human hepatic receptor for high density lipoproteins JM Hoeg, SJ Demosky, SB Edge, RE Gregg, JC Osborne, HB Brewer	Arteriosclerosis (Dallas, Tex.)	1985
Conjugates of colloidal gold with native and acetylated low density lipoproteins for ultrastructural investigations on receptor-mediated endocytosis by cultured human monocyte-derived macrophages AM Mommaas-Kienhuis, JG der Schroeff, MC Wijsman, WT Daems, BJ Vermeer	Histochemistry	1985
Distinct Hepatic Receptors for Low Density Lipoprotein and Apolipoprotein E in Humans JM Hoeg, SJ Demosky, RE Gregg, EJ Schaefer, HB Brewer	Science	1985
HEART-LIVER TRANSPLANTATION IN A PATIENT WITH FAMILIAL HYPERCHOLESTEROLAEMIA TE Starzl, HT Bahnson, RL Hardesty, S Iwatsuki, JC Gartner, DW Bilheimer, BW Shaw, BP Griffith, BJ Zitelli, JJ Malatack, AH Urbach	The Lancet	1984
Heart-liver transplantation in a patient with familial hypercholesterolaemia TE Starzl, DW Bilheimer, HT Bahnson, BW Shaw, RL Hardesty, BP Griffith, S Iwatsuki, BJ Zitelli, JC Gartner, JJ Malatack	Lancet	1984

Citations to this article

Characterization of hepatic low density lipoprotein binding and cholesterol metabolism in normal and homozygous familial hypercholesterolemic subjects.

Characterization of hepatic low density lipoprotein binding and cholesterol metabolism in normal and homozygous familial hypercholesterolemic subjects.

Abstract

Authors

Total citations by year

Citations to this article (53)

Sign up for email alerts