caa a22 4500 469045019 CHVBK 20180323132821.0 cr unu---uuuuu 170328e19920901xx s 000 0 eng 10.1007/BF01800020 doi (NATIONALLICENCE)springer-10.1007/BF01800020 Plasma lipoproteins and monocyte-macrophages in a peroxisome-deficient system: Study of a patient with infantile refsum disease [Elektronische Daten] [H. Mandel, M. Berant, D. Meiron, A. Aizin, J. Oiknine, J. Brook, M. Aviram] Summary: Hypocholesterolaemia in infantile Refsum disease (IRD) may link peroxisomes and lipoprotein metabolism. In our patient, plasma cholesterol levels were reduced to 26% and 29% of control in LDL and HDL fractions, respectively. Plasma apolipoproteins B-100 and A-I levels were 52% and 66% of controls, respectively. In the kindred, plasma cholesterol concentration was 61-73% of controls. The HDL-cholesterol/apo A-I ratios were: patient 0.12; kindred 0.17; controls 0.28. Analysis of the IRD patient's lipoprotein revealed compositional abnormalities in all fractions. The patient's LDL demonstrated a substantial reduction in its lipid-to-protein ratio. Alterations in plasma lipoproteins affect their interaction with macrophages. Upon incubation of the patient's LDL with J-774 macrophages, its cellular uptake, measured as cholesterol esterification rate, was only 66% of a control rate. The abnormal LDL of the IRD patient showed also only 25% of control susceptibility toin vitro oxidation. Studies of cellular cholesterol metabolism in the patient's monocyte-derived macrophages (MDM) showed 57% increased cholesterol esterification rate in comparison to normal MDM. The possible link between lipoprotein abnormalities and monocyte-macrophage cholesterol metabolism is discussed. SSIEM and Kluwer Academic Publishers, 1992 Mandel H. Department of Pediatrics, Rambam Medical Center, and Technion-Faculty of Medicine, 35254, Haifa aut Berant M. Department of Pediatrics, Rambam Medical Center, and Technion-Faculty of Medicine, 35254, Haifa aut Meiron D. Dept of Pediatrics, Emek Central Hospital, Afula aut Aizin A. Laboratory of Clinical Biochemistry, Rambam Medical Center, Haifa aut Oiknine J. Lipid Research Laboratory, Rambam Medical Center and Rappaport Family Institute for Research in Medical Sciences, Technion-Faculty of Medicine, Haifa, Israel aut Brook J. Lipid Research Laboratory, Rambam Medical Center and Rappaport Family Institute for Research in Medical Sciences, Technion-Faculty of Medicine, Haifa, Israel aut Aviram M. Lipid Research Laboratory, Rambam Medical Center and Rappaport Family Institute for Research in Medical Sciences, Technion-Faculty of Medicine, Haifa, Israel aut Journal of Inherited Metabolic Disease Kluwer Academic Publishers 15/5(1992-09-01), 774-784 0141-8955 15:5<774 1992 15 10545 https://doi.org/10.1007/BF01800020 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/BF01800020 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Mandel H. Department of Pediatrics, Rambam Medical Center, and Technion-Faculty of Medicine, 35254, Haifa aut NATIONALLICENCE 700 1- Berant M. Department of Pediatrics, Rambam Medical Center, and Technion-Faculty of Medicine, 35254, Haifa aut NATIONALLICENCE 700 1- Meiron D. Dept of Pediatrics, Emek Central Hospital, Afula aut NATIONALLICENCE 700 1- Aizin A. Laboratory of Clinical Biochemistry, Rambam Medical Center, Haifa aut NATIONALLICENCE 700 1- Oiknine J. Lipid Research Laboratory, Rambam Medical Center and Rappaport Family Institute for Research in Medical Sciences, Technion-Faculty of Medicine, Haifa, Israel aut NATIONALLICENCE 700 1- Brook J. Lipid Research Laboratory, Rambam Medical Center and Rappaport Family Institute for Research in Medical Sciences, Technion-Faculty of Medicine, Haifa, Israel aut NATIONALLICENCE 700 1- Aviram M. Lipid Research Laboratory, Rambam Medical Center and Rappaport Family Institute for Research in Medical Sciences, Technion-Faculty of Medicine, Haifa, Israel aut NATIONALLICENCE 773 0- Journal of Inherited Metabolic Disease Kluwer Academic Publishers 15/5(1992-09-01), 774-784 0141-8955 15:5<774 1992 15 10545 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer