{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,9,20]],"date-time":"2025-09-20T21:54:39Z","timestamp":1758405279314},"reference-count":33,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[1991,4,10]],"date-time":"1991-04-10T00:00:00Z","timestamp":671241600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[1992,9]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>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\u2010100 and A\u2010I levels were 52% and 66% of controls, respectively. In the kindred, plasma cholesterol concentration was 61\u201373% of controls. The HDL\u2010cholesterol\/apo A\u2010I ratios were: patient 0.12; kindred 0.17; controls 0.28.<\/jats:p><jats:p>Analysis of the IRD patient's lipoprotein revealed compositional abnormalities in all fractions. The patient's LDL demonstrated a substantial reduction in its lipid\u2010to\u2010protein ratio. Alterations in plasma lipoproteins affect their interaction with macrophages. Upon incubation of the patient's LDL with J\u2010774 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 to<jats:italic>in vitro<\/jats:italic>oxidation. Studies of cellular cholesterol metabolism in the patient's monocyte\u2010derived macrophages (MDM) showed 57% increased cholesterol esterification rate in comparison to normal MDM. The possible link between lipoprotein abnormalities and monocyte\u2010macrophage cholesterol metabolism is discussed.<\/jats:p>","DOI":"10.1007\/bf01800020","type":"journal-article","created":{"date-parts":[[2005,6,21]],"date-time":"2005-06-21T17:20:51Z","timestamp":1119374451000},"page":"774-784","source":"Crossref","is-referenced-by-count":11,"title":["Plasma lipoproteins and monocyte\u2010macrophages in a peroxisome\u2010deficient system: Study of a patient with infantile refsum disease"],"prefix":"10.1002","volume":"15","author":[{"given":"H.","family":"Mandel","sequence":"first","affiliation":[{"name":"Department of Pediatrics Rambam Medical Center, and Technion\u2010Faculty of Medicine Haifa 35254"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.","family":"Berant","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Rambam Medical Center, and Technion\u2010Faculty of Medicine Haifa 35254"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"D.","family":"Meiron","sequence":"additional","affiliation":[{"name":"Dept of Pediatrics Emek Central Hospital Afula"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A.","family":"Aizin","sequence":"additional","affiliation":[{"name":"Laboratory of Clinical Biochemistry Rambam Medical Center Haifa"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J.","family":"Oiknine","sequence":"additional","affiliation":[{"name":"Lipid Research Laboratory, Rambam Medical Center and Rappaport Family Institute for Research in Medical Sciences Technion\u2010Faculty of Medicine Haifa Israel"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J. G.","family":"Brook","sequence":"additional","affiliation":[{"name":"Lipid Research Laboratory, Rambam Medical Center and Rappaport Family Institute for Research in Medical Sciences Technion\u2010Faculty of Medicine Haifa Israel"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.","family":"Aviram","sequence":"additional","affiliation":[{"name":"Lipid Research Laboratory, Rambam Medical Center and Rappaport Family Institute for Research in Medical Sciences Technion\u2010Faculty of Medicine Haifa Israel"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[1991,4,10]]},"reference":[{"key":"e_1_2_1_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/0006-2944(83)90013-3"},{"key":"e_1_2_1_3_1","doi-asserted-by":"crossref","unstructured":"AviramM(1991)Effect of lipoproteins and platelets on macrophage cholesterol metabolism. 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