Abstract：
:De novo synthesis and turnover of endogenous ceramide in cultured skin fibroblasts from patients affected with Farber lipogranulomatosis were studied by biosynthetical labeling of cellular sphingolipids with [14C]serine. The cellular uptake of [14C]serine and incorporation into de novo synthesized ceramide was similar in normal and Farber fibroblasts, with a half life of newly synthesized ceramide of 2.7 h in normal and diseased cells. Newly synthesized ceramide was found to be channeled directly into biosynthesis of complex sphingolipids rather than contributing to the pool of accumulated ceramide in Farber fibroblasts. The degradation of ceramide generated by the catabolism of complex sphingolipids in Farber cells was greatly delayed compared with control fibroblasts, with differences in the amount of radiolabeled cellular ceramide becoming evident after 6 h chase time. Individual Farber cell lines differed from each other in the amount of accumulated ceramide; however, no correlation was found between ceramide accumulation and residual acid ceramidase activity as determined in vitro. In addition, the amount of radiolabeled sphingomyelin was significantly increased in Farber fibroblasts suggesting a delayed degradation of this compound in this ceramide storage disorder. We propose biosynthetical labeling of endogenous ceramide with [14C]serine, in addition to other established methods, as a highly sensitive and reliable method for the diagnosis of Farber disease, allowing semiquantitative measurement of ceramide accumulation in cultured skin fibroblasts of patients affected with Farber lipogranulomatosis.
journal_name
J Lipid Resjournal_title
Journal of lipid researchauthors
van Echten-Deckert G,Klein A,Linke T,Heinemann T,Weisgerber J,Sandhoff Ksubject
Has Abstractpub_date
1997-12-01 00:00:00pages
2569-79issue
12eissn
0022-2275issn
1539-7262journal_volume
38pub_type
杂志文章abstract：:The ceramide (Cer) and sphingomyelin (SM) species of cultured differentiated rat cerebellar granule cells and human fibroblasts were characterized by electrospray ionization-mass spectrometry. We identified 35 different species of Cer and 18 species of SM in human fibroblasts, and 35 different species of Cer and 9 spe...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：10.1194/jlr.M600344-JLR200
更新日期：2007-02-01 00:00:00
abstract：:Optically active 1- or 3-acyl-sn-glycerols were synthesized from 2,3- or 1,2-isopropylidene-sn-glycerols, respectively. The 2,3- or 1,2-isopropylidene-sn-glycerols were condensed with appropriate long saturated or unsaturated fatty acids and the resulting acyl isopropylidene compounds were treated with dimethylboronbr...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1987-04-01 00:00:00
abstract：:We analysed the sensitivity to beta-adrenoceptor agonists in epididymal adipose cells from rats submitted to a stress protocol previously reported to induce alterations in sensitivity to catecholamines in cardiac tissue from rats. Food intake and body weight were lower, whereas adipocytes basal lipolysis was higher (c...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1999-09-01 00:00:00
abstract：:The intestinal bacterium Eubacterium sp. strain VPI 12708 has been shown to have a bile acid 7 alpha/7 beta-dehydroxylation pathway. A large bile acid inducible (bai) operon encoding at least 9 open reading frames has been cloned and sequenced from this bacterium. The baiE gene from this operon has been subcloned and ...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1996-06-01 00:00:00
abstract：:Dietary lipids are taken up as FAs by the intestinal epithelium and converted by diacylglycerol acyltransferase (DGAT) enzymes into triglycerides, which are packaged in chylomicrons or stored in cytoplasmic lipid droplets (LDs). DGAT1-deficient patients suffer from vomiting, diarrhea, and protein losing enteropathy, i...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：10.1194/jlr.M094201
更新日期：2019-10-01 00:00:00
abstract：:Insulin resistance (IR) is associated with elevated plasma levels of triglyceride-rich lipoproteins (TRLs) of intestinal origin. However, the mechanisms underlying the overaccumulation of apolipoprotein (apo)B-48-containing TRLs in individuals with IR are not yet fully understood. This study examined the relationships...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：10.1194/jlr.M040071
更新日期：2014-01-01 00:00:00
abstract：:Adipogenic differentiation is a highly regulated process that is necessary for metabolic homeostasis and nutrient sensing. The expression of PPARγ and the subsequent activation of adipogenic genes is critical for the process. In this study, we identified lanthionine synthetase C-like protein 2 (LanCL2) as a positive r...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：10.1194/jlr.M085274
更新日期：2018-08-01 00:00:00
abstract：:It was postulated that specialized microdomains of the plasma membrane, consistent with caveolae, might play a role in cholesterol trafficking in intestinal cells. The existence, therefore, of caveolin and the role of detergent-resistant microdomains of the plasma membrane in cholesterol trafficking were investigated ...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1998-10-01 00:00:00
abstract：:Previously, we have shown that lipid-free apoA-I, when incubated with fibroblasts, will produce lipoproteins of pre-alpha mobility (Asztalos, B. F., et al. 1997. Arterioscler. Thromb. Vasc. Biol. 17: 1630-1636). In order to understand the nature of these pre-alpha particles, we further characterized their lipid conten...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1998-08-01 00:00:00
abstract：:Compared with classic essential fatty acid deficiency or the feeding of a fat-free diet, little is known about specific linoleate deficiency in the rat. Carbon recycling into de novo lipogenesis has been reported to be an obligatory feature of linoleate metabolism in the liver, even in extreme linoleate deficiency (LA...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：2000-11-01 00:00:00
abstract：:Chemokines are a family of cytokines inducing cell migration and inflammation. Recent reports have implicated the roles of chemokines in cell differentiation. However, little is known about the functional roles of chemokines in adipocytes. Here, we explored gene expression levels of chemokines and chemokine receptors ...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：10.1194/jlr.M067207
更新日期：2016-10-01 00:00:00
abstract：:Previous studies have indicated that a G to A substitution at position -76 in the gene encoding apolipoprotein A-I (apoA-I) confers increased plasma high density lipoprotein cholesterol (HDL-C). Increased HDL-C may be a direct consequence of the A allele, or may reflect the action of a locus in linkage disequilibrium ...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1994-07-01 00:00:00
abstract：:The rate-limiting step in the transit of dietary fat across the intestinal absorptive cell is its exit from the endoplasmic reticulum (ER) in a specialized ER-to-Golgi transport vesicle, the prechylomicron transport vesicle (PCTV). PCTV bud off from the ER membranes and have unique features; they are the largest ER-de...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：10.1194/jlr.M002758
更新日期：2010-05-01 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：:The relationship between the cholesterol 7 alpha-hydroxylase activity and the pool of free cholesterol in rat liver microsomes was studied under experimental conditions aimed to stimulate (biliary drainage, cholestyramine treatment, and lymphatic drainage) as well as inhibit (chenodeoxycholic acid treatment) bile acid...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1987-03-01 00:00:00
abstract：:Dysbetalipoproteinemia, an uncommon but highly atherogenic mixed hyperlipidemia due to the accumulation of remnants of triglyceride-rich lipoproteins, is characterized by cholesterol-enriched VLDL that migrates in the beta-position on agarose gels. The demonstration of a broad beta-band on agarose gel electrophoresis ...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：10.1194/jlr.d200013-jlr200
更新日期：2003-01-01 00:00:00
abstract：:Apolipoprotein E2 (apoE2)-associated hyperlipidemia is characterized by a disturbed clearance of apoE2-enriched VLDL remnants. Because excess apoE2 inhibits LPL-mediated triglyceride (TG) hydrolysis in vitro, we investigated whether direct or indirect stimulation of LPL activity in vivo reduces the apoE2-associated hy...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：10.1194/jlr.M800009-JLR200
更新日期：2008-05-01 00:00:00
abstract：:Some commercial preparations of common natural conjugated bile salts contain impurities (e.g., amines, lipids, and calcium) that are likely to affect their physicochemical properties. A method was developed for purifying commercial preparations of sodium salts of glycine- and taurine-conjugated bile acids. The method ...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1995-12-01 00:00:00
abstract：:In the present study, the effects of taxifolin, a plant flavonoid, on lipid, apolipoprotein B (apoB), and apolipoprotein A-I (apoA-I) synthesis and secretion were determined in HepG2 cells. Pretreatment of cells with (+/-)-taxifolin led to an inhibition of cholesterol synthesis in a dose- and time-dependent manner, wi...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：2000-12-01 00:00:00
abstract：:Gel-permeation chromatography on Sephadex LH-20, using ethanol as eluent, permits the resolution of neutral hydroxy lipids according to molecular size. The influence of molecular shape, functional groups, chain lengths, and degree of unsaturation, as well as the effect of the eluent on the elution pattern are discusse...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1970-03-01 00:00:00
abstract：:Human high density lipoprotein enriched in free cholesterol was obtained by exposing the lipoprotein to lipid dispersions having a free cholesterol/lecithin molar ratio greater than two. The metabolism of cholesterol was studied in tissue culture cells exposed to normal and cholesterol-enriched lipoproteins. Incubatio...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1978-03-01 00:00:00
abstract：:We investigated, in the present study, the role of reactive oxygen intermediates (ROI) in the control of macrophage lipoprotein lipase (LPL) secretion. Exposure of murine macrophages to increasing concentrations of hydrogen peroxide (H2O2) resulted in enhanced basal LPL production and mRNA levels. The increase of LPL ...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1996-04-01 00:00:00
abstract：:Experimental approaches involving the perfusion of tissues and organs offer the advantage of improved physiological relevance over the use of isolated tissues or cells while at the same time being much more controlled and tissue-specific than studies in vivo. Nevertheless, there have been few metabolic studies perform...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：10.1194/jlr.D500016-JLR200
更新日期：2005-08-01 00:00:00
abstract：:Pregnancy is associated with increases in plasma total cholesterol (TC) and triglycerides (TG). Individuals with decreased LPL activity have a mild form of hypertriglyceridemia. Variations in the apolipoprotein E (apoE) gene have been associated with increases in plasma TG in addition to differences in plasma TC, LDL ...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：2001-11-01 00:00:00
abstract：:Cholesteryl ester storage disease (CESD) and Wolman disease (WD) are both autosomal recessive disorders associated with reduced activity and genetic defects of lysosomal acid lipase (LAL). The strikingly more severe course of WD is caused by genetic defects of LAL that leave no residual enzymatic activity. Mutations a...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1996-08-01 00:00:00
abstract：:We evaluated the role of sterol-regulatory element binding protein (SREBP)-1c/peroxisome proliferator activated receptor-gamma (PPARgamma) pathway on heart lipotoxicity in patients with metabolic syndrome (MS) and aortic stenosis (AS). Echocardiographic parameters of heart function and structural alterations of LV spe...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：10.1194/jlr.P900032-JLR200
更新日期：2009-11-01 00:00:00
abstract：:A molecular model was built for human lecithin:cholesterol acyltransferase (LCAT) based upon the structural homology between this enzyme and lipases (Peelman et al. 1998. Prot. Sci. 7: 585-597). We proposed that LCAT belongs to the alpha/beta hydrolase fold family, and that the central domain of LCAT consists of a mix...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1999-01-01 00:00:00
abstract：:Seven Z-octadecenoic acids having the double bond located in positions 6Z to 13Z were photooxidized. The resulting hydroperoxy-E-octadecenoic acids [HpOME(E)] were resolved by chiral phase-HPLC-MS, and the absolute configurations of the enantiomers were determined by gas chromatographic analysis of diastereoisomeric d...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：10.1194/jlr.M018259
更新日期：2011-11-01 00:00:00
abstract：:Genetic polymorphisms of apolipoprotein A-IV (apoA-IV) have been detected by isoelectric focusing of serum proteins. Because genetic variation in apoA-IV has significant effects on lipid risk factors, we used restriction enzyme isoform genotyping (restriction isotyping) to determine apoA-IV isoform genotypes at the DN...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1991-09-01 00:00:00
abstract：:An elevated plasma concentration of lipoprotein (a) [Lp(a)] is an independent risk factor for cardiovascular disease. Life style modification and currently available drugs either fail to effectively lower plasma Lp(a) levels or do not result in clinical benefit. However, lipoprotein apheresis is very efficient in decr...
journal_title：Journal of lipid research
pub_type： 杂志文章,评审
doi：10.1194/jlr.R056549
更新日期：2016-10-01 00:00:00
