Abstract：
:Familial hypobetalipoproteinemia (FHBL) exists in three forms: a) FHBL genetically linked to truncated forms of apolipoprotein B (apoB); b) FHBL linked to the apoB gene but with no apoB truncations; and c) FHBL not linked to the apoB gene. Mean production rate (PR) of apoB-100 in FHBL subjects heterozygous for apoB truncations is approximately 30% of normal. In a 49-member D-kindred (FHBL phenotype defined as apoB < 40 mg/dl), no apoB truncations were detectable either by immunoblotting of plasma or by sequencing of relevant stretches of the apoB gene. Herein we report on the kinetic parameters of apoB-100-containing lipoproteins in four affected members of the D-kindred, and compare their kinetic values to 14 normal subjects, and 8 previously reported FHBL subjects heterozygous for various truncated forms of apoB. After an 8-h primed intravenous infusion of [13C]-leucine, enrichments of apoB-100 were assessed by gas chromatography-mass spectrometry and kinetic parameters were calculated by multicompartmental modeling. The affected members of the D-kindred had similar very low, intermediate, and low density lipoprotein (VLDL, IDL, and LDL) PRs as normal controls, but their fractional catabolic rates (FCR) for VLDL and LDL were approximately 2 and 3 times higher, respectively, than those of normals. By contrast in apoB truncation subjects, apoB-100 PRs were uniformly reduced, while apoB-100 FCRs were similar to normals. Thus, diverse physiologic mechanisms are responsible for the low apoB levels in these two different, genetically determined forms of FHBL.
journal_name
J Lipid Resjournal_title
Journal of lipid researchauthors
Latour MA,Patterson BW,Pulai J,Chen Z,Schonfeld Gsubject
Has Abstractpub_date
1997-03-01 00:00:00pages
592-9issue
3eissn
0022-2275issn
1539-7262journal_volume
38pub_type
杂志文章abstract：:Vitamin E is a naturally occurring fat-soluble antioxidant which has been proposed as a treatment for both primary and secondary protection against cardiovascular (CV) events. Promising data from observational epidemiological studies associating higher vitamin E dietary intake with lower risk of CV events have not bee...
journal_title：Journal of lipid research
pub_type： 杂志文章,评审
doi：10.1194/jlr.R026641
更新日期：2013-09-01 00:00:00
abstract：:A facile procedure is described for the preparation of [14C]epicholesterol from [14C]cholesterol. Cholesterol is first converted to cholesteryl mesylate, which is treated with cesium acetate and 18-crown-6 in refluxing toluene to give epicholesteryl acetate. The latter is hydrolyzed, without isolation, with potassium ...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1990-01-01 00:00:00
abstract：:Oxylipins are potent lipid mediators involved in a variety of physiological processes. Their profiling has the potential to provide a wealth of information regarding human health and disease and is a promising technology for translation into clinical applications. However, results generated by independent groups are r...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：10.1194/jlr.RA120000991
更新日期：2020-11-01 00:00:00
abstract：:Recent studies from our laboratory (Yancey, P.G., and R. W. St. Clair. 1992. Arterioscler. Thromb. 12: 1291-1304) have shown that cultured peritoneal macrophages from White Carneau (WC) pigeons clear cholesteryl esters at a slower rate than do macrophages from Show Racer (SR) pigeons (9% per 24 h vs. 42% per 24 h, res...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1994-12-01 00:00:00
abstract：:Liver and kidney microsomes have been found to catalyze a rapid decarboxylation of retinoic acid in vitro. The reaction requires NADPH and Fe(2+), and is further stimulated by the presence of pyrophosphate. Thiamine pyrophosphate contained sufficient iron as an impurity to provide strong enhancement of the reaction in...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1968-07-01 00:00:00
abstract：:Rat lymph chylomicrons were treated with rat heparin-releasable hepatic lipase (HL) or with bovine milk lipoprotein lipase (LPL). The ability of the resulting particles to be taken up by the liver in vivo was assessed following their infusion into the portal vein of partially hepatectomized animals. The following obse...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1988-08-01 00:00:00
abstract：:Apolipoprotein E plays a critical role in plasma lipoprotein clearance. Peptide models of a highly conserved, N-terminal domain of this protein have been shown to increase the binding of low density lipoprotein (LDL) to fibroblast cell surfaces independently of the low density lipoprotein receptor. Here we provide dat...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1999-04-01 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：:Cardiac triacylglycerol (TG) catabolism critically depends on the TG hydrolytic activity of adipose triglyceride lipase (ATGL). Perilipin 5 (Plin5) is expressed in cardiac muscle (CM) and has been shown to interact with ATGL and its coactivator comparative gene identification-58 (CGI-58). Furthermore, ectopic Plin5 ex...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：10.1194/jlr.M034710
更新日期：2013-04-01 00:00:00
abstract：:Niemann-Pick disease, type C (NP-C), often associated with Niemann-Pick disease, type C1 (NPC1) mutations, is a cholesterol-storage disorder characterized by cellular lipid accumulation, neurodegeneration, and reduced steroid production. To study NPC1 function in vivo, we cloned zebrafish npc1 and analyzed its gene ex...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：10.1194/jlr.M012377
更新日期：2011-07-01 00:00:00
abstract：:Lymph chylomicrons of different sizes are known to be cleared at different rates, but the underlying mechanism for this effect has not been resolved. To investigate the differences in clearance rates between small and large particles, chylomicron-like lipid emulsions labeled with radioactive triolein and cholesteryl o...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1996-12-01 00:00:00
abstract：:The rate of lipolytic enzyme-catalyzed reactions yielding water-soluble products can be measured by isobaric titration. The method is based upon the measurement of the amount of substrate that must be added to a monalayer to maintain constant surface pressure during the course of the enzymatic reaction. The rate const...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1975-01-01 00:00:00
abstract：:The objective of this study was to investigate the mobilization of individual fatty acids from human subcutaneous adipose tissue in vivo. Concentrations of individual non-esterified fatty acids were measured in arterialized plasma and in the venous drainage from subcutaneous abdominal adipose tissue in eight normal su...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1996-09-01 00:00:00
abstract：:In a previously described method for determining the activity of cerebroside galactosidase, the enzyme preparation was incubated with an emulsion of cerebroside which had been labeled in the galactose moiety. The liberated galactose was separated from the emulsion by liquid-liquid partitioning, but the presence of det...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1971-03-01 00:00:00
abstract：:Lipoprotein lipase (LPL) and hepatic lipase (HL) mediate the hydrolysis of triglycerides and phospholipids present in circulating lipoprotein particles and are essential for normal lipid metabolism. Both enzymes have a similar primary amino acid structure and share requirements for intact catalytic, lipid binding, and...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1993-08-01 00:00:00
abstract：:LDL-associated phospholipids (PLs) may be transferred into platelets. In this work, we characterized the role of VLDLs as PL donors. VLDL transferred radiolabeled PLs to platelets in a temperature- and concentration-dependent manner. LPL stimulated this process through its action on VLDL lipolysis, because it was abol...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：10.1194/jlr.M500209-JLR200
更新日期：2006-02-01 00:00:00
abstract：:Total serum free fatty acids (FFAt) levels provide an important measure of the physiologic state. Most of the FFA in serum is bound to albumin; a small portion, however, is unbound. This study presents the first measurements of serum unbound free fatty acid (FFAu) concentrations. These measurements were made possible ...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1995-02-01 00:00:00
abstract：:Alcoholic liver disease (ALD) is the most prevalent type of chronic liver disease with significant morbidity and mortality worldwide. ALD begins with simple hepatic steatosis and progresses to alcoholic steatohepatitis, fibrosis, and cirrhosis. The severity of hepatic steatosis is highly associated with the developmen...
journal_title：Journal of lipid research
pub_type： 杂志文章,评审
doi：10.1194/jlr.R119000547
更新日期：2020-04-01 00:00:00
abstract：:AY 9944 is a novel inhibitor of cholesterol biosynthesis which appears to act on the Delta 7-reductase enzyme, interfering with the conversion of 7-dehydrocholesterol to cholesterol. In all tissues studied, with the exception of the lens, there was a reduction in cholesterol and a marked accumulation of 7-dehydrochole...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1966-01-01 00:00:00
abstract：:The basic protein protamine strongly inhibited hydrolysis of triolein emulsified with soybean phosphatidylcholine (PC) by pancreatic and carboxylester lipases; 10 micrograms/ml protamine, about 1000 times lower than the concentration of bovine serum albumin for the same effect, inhibited triolein hydrolysis completely...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1996-07-01 00:00:00
abstract：:The apoproteins of serum lipoproteins and of lipoproteins present in a nonrecirculating perfusate of rat liver were compared by immunochemical, gel electrophoretic, and solubility techniques. Serum and perfusate very low density lipoprotein apoprotein composition were not different. No evidence for the presence of a l...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1976-01-01 00:00:00
abstract：:We have previously shown that expression of a human apoE cDNA in J774 macrophages enhances cholesterol efflux to HDL3. We have also shown that endogenous apoE expression produces a cell surface pool of apoE associated with proteoglycans. In this series of experiments, we first demonstrate the presence of a cell surfac...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1998-02-01 00:00:00
abstract：:Activation of cultured hepatic stellate cells correlated with an enhanced expression of proteins involved in uptake and storage of fatty acids (FA translocase CD36, Acyl-CoA synthetase 2) and retinol (cellular retinol binding protein type I, CRBP-I; lecithin:retinol acyltransferases, LRAT). The increased expression of...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：10.1194/jlr.M200376-JLR200
更新日期：2003-02-01 00:00:00
abstract：:To identify novel fatty acid diol synthases, putative candidate sequences from Penicillium species were analyzed, and hydroxy fatty acid production by crude Penicillium enzyme extracts was assessed. Penicillium chrysogenum was found to produce an unknown dihydroxy fatty acid, a candidate gene implicated in this produc...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：10.1194/jlr.M061341
更新日期：2016-02-01 00:00:00
abstract：:Endothelial dysfunction is a key step in atherosclerosis development. Our recent studies suggested that oxLDL-induced increase in endothelial stiffness plays a major role in dyslipidemia-induced endothelial dysfunction. In this study, we identify oxysterols, as the major component of oxLDL, responsible for the increas...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：10.1194/jlr.M027102
更新日期：2012-07-01 00:00:00
abstract：:The technique of short bed--continuous development chromatography has been utilized to increase the separation of glycosphingolipids on high performance thin-layer chromatography plates. The theoretical goal of increasing separation of bands by decreasing solvent strength was achieved within a practical time span as a...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1986-01-01 00:00:00
abstract：:After stereospecific oxidation, trimethylsilylated methyl esters of mono- and diunsaturated fatty acids were analyzed by combined gas-liquid chromatography-chemical ionization mass spectrometry. The positions of original double bonds were deduced from the fragment ions produced by the cleavage of the carbon-carbon bon...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1978-02-01 00:00:00
abstract：:To study the role of phospholipids in the lipophorin structure, they were removed by phospholipase A2 treatment. Fluorescence lifetimes and accessibility to acrylamide quenching of different diphenylhexatrienyl derivatives, which were used as analogues of the different lipid components, indicate a surface localization...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1996-10-01 00:00:00
abstract：:Previous studies have shown that antibodies to cubulin, a receptor on the yolk sac that binds high density lipoproteins (HDL) and cobalamin, induce fetal abnormalities. Mice with markedly low concentrations of plasma HDL-cholesterol (HDL-C) give birth to healthy pups, however. To establish whether maternal HDL-C has a...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：2000-03-01 00:00:00
abstract：:In vivo single-voxel magnetic resonance spectroscopy (MRS) at 4.7T and ex vivo high-resolution proton magnetic resonance spectroscopy (HR-NMR) at 500 MHz were used to study the composition of adipose tissues in Zucker obese and Zucker lean rats. Lipid composition was characterized by unsaturation and polyunsaturation ...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：10.1194/jlr.M011825
更新日期：2011-02-01 00:00:00
