Abstract：
:This paper presents a case of typical hyperlipoproteinemia type I in a young woman. Her serum triglycerides varied between 2 and 90 mmol/l and she had substantial amounts of apolipoprotein B-48 in fasting plasma. She had no detectable lipoprotein lipase (LPL) activity in post-heparin plasma (less than 0.2 percent of normal). Southern blot analysis suggested no major defect in her LPL gene and Northern blot analysis of adipose tissue RNA showed normal-sized LPL-mRNA. A 2-h [35S]methionine incorporation experiment with adipose tissue pieces in vitro showed that she produced normal-sized LPL and had LPL catalytic activity in the tissue. The amounts were, however, only 5-10% of control. No detectable LPL radioactivity or catalytic activity was released from patient tissue even in the presence of heparin in the incubations. Immunofluorescent staining of adipose tissue biopsies from the patient showed LPL immunoreactivity only in adipocytes and little or none within the capillaries. Treatment of immunoprecipitated labeled LPL with endoglycosidase H showed that the oligosaccharide chains on her enzyme were of the high-mannose type and not processed as in controls. Taken together the data suggest that the patient synthesizes a relatively normal LPL protein which is core-glycosylated and folded into active enzyme as in normal subjects, but is not effectively transported via the Golgi to the cell surface.
journal_name
J Lipid Resjournal_title
Journal of lipid researchauthors
Fager G,Semb H,Enerbäck S,Olivecrona T,Jonasson L,Bengtsson-Olivecrona G,Camejo G,Bjursell G,Bondjers Gsubject
Has Abstractpub_date
1990-07-01 00:00:00pages
1187-97issue
7eissn
0022-2275issn
1539-7262journal_volume
31pub_type
杂志文章abstract：:For the last 30 years fibrates have been widely prescribed to treat human dyslipidemia. However, the primary mechanism by which they lower plasma lipid levels is still unknown. Studies with transgenic mice have suggested that changes in apoC-III expression levels have a dramatic influence on plasma triglyceride levels...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1995-12-01 00:00:00
abstract：:The direct effect of apolipoprotein (apo) E on cholesteryl ester transfer protein (CETP) activity was studied using lipoproteins from a subject with apoE deficiency (Atherosclerosis. 1991. 88: 15-20) as a model system. The transfer of cholesteryl ester (CE) from discoidal bilayer particles (DBP) to very low density li...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1993-02-01 00:00:00
abstract：:Major neutral glycosphingolipid components were extracted from Sporothrix schenckii, a dimorphic fungus exhibiting a hyphal saprophytic phase and a yeast parasitic phase responsible for chronic mycotic infections in mammalian hosts. These components, one from the mycelial form and two from the yeast form, were purifie...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：2000-05-01 00:00:00
abstract：:Conditions under which apolipoprotein (apo) A-I dissociates from human high density lipoproteins (HDL) during incubation in vitro have been investigated. Dissociation of apoA-I was demonstrated by non-denaturing gradient gel electrophoresis followed by immunoblotting for apoA-I and by size-exclusion chromatography. It...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1994-07-01 00:00:00
abstract：:In a randomized, cross-over feeding trial involving 10 men with polygenic hypercholesterolemia, a control, Mediterranean-type cholesterol-lowering diet, and a diet of similar composition in which walnuts replaced approximately 35% of energy from unsaturated fat, were given for 6 weeks each. Compared with the control d...
journal_title：Journal of lipid research
pub_type： 临床试验,杂志文章,随机对照试验
doi：
更新日期：2001-12-01 00:00:00
abstract：:Hyperlipoproteinemia contributes both to kidney disease progression and the development of atherosclerosis. Elevated high density lipoprotein cholesterol and apolipoprotein A-I (apoA-I) serum levels are independent factors protective against the atherosclerotic process. We examined the effects in a transgenic rat mode...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1995-07-01 00:00:00
abstract：:Several studies indicate that cholesterol esterification is deregulated in cancers. The present study aimed to characterize the role of cholesterol esterification in proliferation and invasion of two tumor cells expressing an activated cholecystokinin 2 receptor (CCK2R). A significant increase in cholesterol esterific...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：10.1194/jlr.M800668-JLR200
更新日期：2009-11-01 00:00:00
abstract：:Electrospray ionization mass spectrometry has previously been used to probe qualitative changes in the phospholipid cardiolipin (CL), but it has rarely been used in a quantitative manner. We assessed changes in the amount of individual molecular species of cardiac CL present in a model of congestive heart failure usin...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：10.1194/jlr.M500031-JLR200
更新日期：2005-06-01 00:00:00
abstract：:Elongation of very long chain fatty acid-like family member 6 (ELOVL6) is a fatty acyl elongase that performs the initial and rate-limiting condensing reaction required for microsomal elongation of long-chain fatty acids. Our previous in vitro studies suggested that ELOVL6 elongated long-chain saturated fatty acids an...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：10.1194/jlr.M054353
更新日期：2014-12-01 00:00:00
abstract：:A group of polyunsaturated fatty acids called conjugated linoleic acids (CLAs) are found in ruminant products, where the most common isomers are cis9, trans11 (c 9,t11) and trans10, cis12 (t10,c12) CLA. A crude mixture of these isomers has been shown in animal studies to alter body composition by a reduction in body f...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：10.1194/jlr.M300120-JLR200
更新日期：2003-08-01 00:00:00
abstract：:Ecdysteroids are potent developmental regulators that control molting, reproduction, and stress response in arthropods. In developing larvae, picogram quantities of individual ecdysteroids and their conjugated forms are present along with milligrams of structural and energy storage lipids. To enhance the specificity a...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：10.1194/jlr.D035949
更新日期：2013-08-01 00:00:00
abstract：:The interaction of hepatic lipase (HL) with heparan sulfate is critical to the function of this enzyme. The primary amino acid sequence of HL was compared to that of lipoprotein lipase (LPL), a related enzyme that possesses several putative heparin-binding domains. Of the three putative heparin-binding clusters of LPL...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：2000-02-01 00:00:00
abstract：:The initial studies of the metabolism of arachidonic acid (AA) by the cytochrome P450 (P450) hemeproteins sought to: a) elucidate the roles for these enzymes in the metabolism of endogenous pools of the FA, b) identify the P450 isoforms involved in AA epoxidation and ω/ω-1 hydroxylation, and c) explore the biological ...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：10.1194/jlr.R087882
更新日期：2018-11-01 00:00:00
abstract：:We have previously observed that the Ya subunit-containing glutathione (GSH) S-transferases from rat liver exhibit a common high affinity binding site for lithocholic acid, bilirubin, and sulfobromophthalein (BSP) (1984. J. Lipid Res. 25: 1177-1183). Subsequently we found that cholic acid and its amidates bound to a s...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1988-03-01 00:00:00
abstract：:The amphipathic alpha helix is an often-encountered secondary structural motif in biologically active peptides and proteins. An amphipathic helix is defined as an alpha helix with opposing polar and nonpolar faces oriented along the long axis of the helix. In a recent review article we grouped amphipathic helixes into...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1992-02-01 00:00:00
abstract：:In this work, we investigated the impact of testosterone deficiency and cholesteryl ester transfer protein (CETP) expression on lipoprotein metabolism and diet-induced atherosclerosis. CETP transgenic mice and nontransgenic (nTg) littermates were studied 4 weeks after bilateral orchidectomy or sham operation. Castrate...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：10.1194/jlr.M600135-JLR200
更新日期：2006-07-01 00:00:00
abstract：:It was the aim of this study to i) compare the effects of glucose and other hexoses with that of oleate on secretion of apolipoproteins (apos) A-I and B by HepG2 cells, and ii) document the effect of various metabolic inhibitors on the secretion of these apos in the absence or presence of extra glucose/oleate. i) The ...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1995-08-01 00:00:00
abstract：:Triglyceride-rich lipoproteins derived from ten normo- and hyperlipidemic apoE-2 homozygotes were analyzed for their composition, beta-VLDL content, and their ability to induce cholesteryl ester storage in macrophages. In six of these probands apoE sequence analysis revealed that the cysteine residues were at position...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1985-03-01 00:00:00
abstract：:Various detergents can be used to dissolve phospholipids, resulting in very narrow 31PNMR resonances. The resonances are well resolved, allowing identification and quantitative analysis of phospholipids in a mixture. The chemical shift depends strongly on pH, reflecting changes in the state of ionization of the phosph...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1979-03-01 00:00:00
abstract：:Hepatic free cholesterol levels are influenced by cholesterol synthesis and ester formation, which, in turn, might regulate cholesterol secretion into bile and plasma. We manipulated the rates of hepatic cholesterol synthesis and esterification and measured biliary and very low density lipoprotein (VLDL) cholesterol s...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1992-11-01 00:00:00
abstract：:A synthetic analog of glucocerebroside, N-hexyl-O-glucosyl sphingosine, was found to inhibit the glucosidase in rat spleen that hydrolyzes glucocerebroside. At a concentration of 1 micro m, the analog inhibited the enzyme by 48%. The mode of action appeared to be competitive, probably aided by tight binding of the ami...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1973-03-01 00:00:00
abstract：:3-Hydroxy-3-methylglutaryl coenzyme A (HMG-CoA) reductase, the enzyme catalyzing the rate-limiting step in cholesterol biosynthesis, exists in one active (dephosphorylated) and one inactive (phosphorylated) form in liver microsomes obtained from several animal species. The present study was undertaken in order to dete...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1984-11-01 00:00:00
abstract：:The de novo ceramide synthesis pathway is essential to human biology and health, but genetic influences remain unexplored. The core function of this pathway is the generation of biologically active ceramide from its precursor, dihydroceramide. Dihydroceramides have diverse, often protective, biological roles; converse...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：10.1194/jlr.P094433
更新日期：2019-09-01 00:00:00
abstract：:Diet-induced changes in high density lipoprotein (HDL) density and size were studied in patas monkeys. When the animals were switched from a moderate fat-low cholesterol diet to a high fat-high cholesterol (HFHC) diet, the plasma apoA-I levels increased initially in all of the animals. The apoA-I levels remained eleva...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1984-09-01 00:00:00
abstract：:Cholesterol-3-(3)H,4-(14)C was injected intravenously in man and its transformation to cholestanol was studied. From the (3)H: (14)C ratios in cholestanol isolated from blood, evidence for the participation of a ketonic intermediate in the conversion was obtained. In a second subject given cholestanol-3-(3)H,4-(14)C t...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1967-01-01 00:00:00
abstract：:Significant amounts of ursodeoxycholic acid (UDCA) used for the treatment of patients with primary biliary cirrhosis (PBC) become epimerized at C-3 to isoUDCA. We investigated the metabolism of isoUDCA and a possible pharmacologic effect in five patients (51.4 +/- 5.8 years old; 3 females, 2 males) with PBC and persis...
journal_title：Journal of lipid research
pub_type： 临床试验,杂志文章
doi：
更新日期：2001-05-01 00:00:00
abstract：:LCAT plays a key role in the maturation of HDL, as evidenced by low HDL-cholesterol levels in carriers of deleterious mutations in LCAT. However, the role of LCAT in atherosclerosis is unclear. We set out to study this in a prospective study. Plasma LCAT levels, which strongly correlate with LCAT activity, were measur...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：10.1194/P900038-JLR200
更新日期：2010-02-01 00:00:00
abstract：:Apolipoprotein (apo) B-100, the protein constituent of low density lipoproteins (LDL), is the determinant responsible for LDL binding to the apoB,E(LDL) receptor on cells. The current study was designed to identify the region(s) of apoB-100 that interact with the apoB,E(LDL) receptor. Apolipoprotein B-100 was fragment...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1987-12-01 00:00:00
abstract：:Analysis of plasma nonesterified fatty acids (NEFA) by gas-liquid chromatography requires procedures that are both lengthy and cumbersome. A 45-min direct methylation procedure was carried out at 24-29 degrees C on 150 microliter of plasma added with an internal standard in 5.0 ml of methanol-acetyl chloride 50:1 (v/v...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1988-02-01 00:00:00
abstract：:The central region of apolipoprotein A-I (apoA-I), spanning residues 143--165, has been implicated in lecithin:cholesterol acyltransferase (LCAT) activation and also in high density lipoprotein (HDL) structural rearrangements. To examine the role of individual amino acids in these functions, we constructed, overexpres...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：2001-03-01 00:00:00
