Abstract:
:Glycolipids were isolated from the brain of a patient with a myoclonic variant of late infantile amaurotic idiocy. There was an abnormal glycolipid pattern in gray and white matter. The observed high concentration of gangliosides was due to a uniform accumulation of all four major gangliosides and was not limited to one species such as ganglioside A(1), as in Tay-Sachs disease, or ganglioside A(2), as in gangliosidosis-Gm1. Two additional stored substances were identified as ceramide lactoside and ceramide tetrahexoside. Partial and total hydrolysis of these ceramide hexosides revealed that their ceramide moiety is identical with the ceramide portion of gangliosides. The sequence of hexoses in the carbohydrate chain of the ceramide dihexoside and ceramide tetrahexoside further suggests a metabolic and chemical relation to gangliosides. Some implications of these findings for the theories of the metabolic defects in gangliosidoses are discussed.
journal_name
J Lipid Resjournal_title
Journal of lipid researchauthors
Bartsch GGsubject
Has Abstractpub_date
1970-05-01 00:00:00pages
241-7issue
3eissn
0022-2275issn
1539-7262journal_volume
11pub_type
杂志文章abstract::Most mammalian neoplasms have a defect in ether lipid content manifested by the presence of abnormally large quantities of 0-alkyl glyceryl ethers, in contrast to normal tissues in which the alk-1-enyl structure predominates. These lipids are for the most part structural. The manner in which tumor cell plasma membrane...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1978-01-01 00:00:00
abstract::Mammalian spermatogenesis is a complex developmental program in which a diploid progenitor germ cell transforms into highly specialized spermatozoa. One intriguing aspect of sperm production is the dynamic change in membrane lipid composition that occurs throughout spermatogenesis. Cholesterol content, as well as its ...
journal_title:Journal of lipid research
pub_type: 杂志文章,评审
doi:10.1194/jlr.R032326
更新日期:2013-01-01 00:00:00
abstract::Paraoxonases (PONs) are a small family of antioxidant enzymes whose antiatherogenic activity is well known. The aim of the present study was the evaluation of the effects of moderate aerobic training on their expression using a rat model. In order to discriminate between PON1 and PON3 enzymatic activity, we took advan...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:10.1194/jlr.M800493-JLR200
更新日期:2009-10-01 00:00:00
abstract::Rat liver microsomes were incubated with the monofunctional aminoreagent fluorescamine. Although the probe easily penetrated the membranes, two pools of phosphatidylethanolamine (PE) could be detected. The first pool rapidly reacted with the probe and comprised 80% of the total PE. The second pool exhibited a very slo...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1986-07-01 00:00:00
abstract::The hydrolysis of HDL phospholipids (PL) and glycerides by hepatic lipase (HL) has been investigated in native and reconstituted HDL particles (Lp2A-I). Fasting, normolipidemic HDL exhibit total lipid hydrolytic rates of between 10 and 36 nM FA/h per microM PL. Of the total fatty acids liberated with HDL3 only 1% are ...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1997-11-01 00:00:00
abstract::After crossing floxed stearoyl-CoA desaturase-1 (Scd1fl/fl) mice with LDL receptor-null (ldlr-/-) mice, and then Villin Cre (VilCre) mice, enterocyte Scd1 expression in Scd1fl/fl/ldlr-/-/VilCre mice was reduced 70%. On Western diet (WD), Scd1fl/fl/ldlr-/- mice gained more weight than Scd1fl/fl/ldlr-/-/VilCre mice (P <...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:10.1194/jlr.M083527
更新日期:2018-10-01 00:00:00
abstract::Apolipoprotein AV (apoAV) overexpression causes a decrease in plasma triglyceride (TG) levels, while deficiency of apoAV causes hypertriglyceridemia in both men and mice. However, contrary to what would be expected, plasma apoAV and TG levels in humans are positively correlated. To address this apparent paradox, we de...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:10.1194/jlr.M800551-JLR200
更新日期:2009-05-01 00:00:00
abstract::H-Rev107 is a protein that was previously cloned as a negative regulator of proto-oncogene Ras and classified as a class II tumor suppressor. Its structural similarity to lecithin retinol acyltransferase and Ca2+-independent phosphatidylethanolamine (PE) N-acyltransferase led us to analyze H-Rev107 as an enzyme involv...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:10.1194/jlr.M800453-JLR200
更新日期:2009-04-01 00:00:00
abstract::Hydroxy-3-methyl-glutaryl-CoA reductase (HMGR) is the rate-controlling enzyme of cholesterol synthesis, and owing to its biological and pharmacological relevance, researchers have investigated several compounds capable of modulating its activity with the hope of developing new hypocholesterolemic drugs. In particular,...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:10.1194/jlr.M011817
更新日期:2011-05-01 00:00:00
abstract::High plasma cholesterol levels are found in several metabolic disorders and their reductions are advocated to reduce the risk of atherosclerosis. A way to lower plasma lipids is to curtail lipoprotein production; however, this is associated with steatosis. We previously showed that microRNA (miR)-30c lowers diet-induc...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:10.1194/jlr.M081299
更新日期:2018-01-01 00:00:00
abstract::The extent of hypercholesterolemia varies considerably in patients with familial hypercholesterolemia (FH). We hypothesized that the variability of the FH phenotype might be partly explained by variation in proprotein convertase subtilisin kexin type 9 (PCSK9) activity. Individuals between 18 and 53 years of age who h...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:10.1194/jlr.P023994
更新日期:2012-05-01 00:00:00
abstract::Mature endothelial lipase (EL) is a 68 kDa glycoprotein. In HepG2 cells infected with adenovirus encoding human EL, the mature EL was detectable in the cell lysates and heparin-releasable fractions. In contrast, cell media of these cells contained two EL fragments: an N-terminal 40 kDa fragment and a C-terminal 28 kDa...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:10.1194/jlr.M400500-JLR200
更新日期:2005-05-01 00:00:00
abstract::Adipose tissue development was studied histochemically and histologically in fetuses from lean and obese sows. At 110 days of gestation, fetuses were removed from Ossabaw (obese-feral) sows and from sows selected for high backfat (obese-domestic) and for low backfat (lean) thickness. Body weights were similar for lean...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1983-03-01 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
abstract::A strategy has been developed for archaebacterial lipid analysis which provides three times the information to describe archaebacterial isolates and is compatible with simultaneous eubacterial/eukaryotic lipid analysis of environmental samples. Eubacterial and micro-eukaryotic biomass, community structure, and nutriti...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1991-04-01 00:00:00
abstract::Xanthophyllomyces dendrorhous is a basidiomycete yeast known as a natural producer of astaxanthin, a carotenoid of commercial interest because of its antioxidant properties. Recent studies indicated that X. dendrorhous has a functional SREBP pathway involved in the regulation of isoprenoid compound biosynthesis, which...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:10.1194/jlr.RA119000431
更新日期:2020-02-01 00:00:00
abstract::A simple, rapid, inexpensive method for isolating disaturated phosphatidylcholine from adult rat lung has been devised. Total lipids are reacted with osmium tetroxide dissolved in carbon tetrachloride, and the disaturated phosphatidylcholine is isolated on a column of neutral alumina. More than 99% of the fatty acids ...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1976-05-01 00:00:00
abstract::Smith-Lemli-Opitz syndrome (SLOS) is a genetic disorder characterized by low plasma cholesterol and high 7-dehydrocholesterol (7-DHC). Synthesis of cholesterol and 7-DHC and its metabolites is regulated by HMG-CoA reductase, whose activity can be measured by 24-h excretion of its product mevalonate. We devised a simpl...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:10.1194/jlr.m200163-jlr200
更新日期:2002-10-01 00:00:00
abstract::Fatty acids (FA) that are utilized for triglyceride (TG) synthesis in the liver and principally from two sources: FA synthesized de novo in the liver and preformed FA. We have measured the contribution from the two sources to very low density lipoprotein (VLDL) TG synthesis individually for palmitate, oleate, stearate...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1998-06-01 00:00:00
abstract::Apolipoprotein E (apoE) polymorphism is a genetic determinant of plasma lipid levels and of coronary heart disease (CHD) risk. We determined the apoE phenotypes and plasma lipid levels in 1577 youths aged 3 to 18 years in 1980. The subjects were randomly selected from five areas of Finland. ApoE phenotyping was perfor...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1990-03-01 00:00:00
abstract::A method has been developed for rapidly quantitating the cholesterol concentration of normal and certain variant lipoproteins in a large number of patients (over 240 in one week). The method employs a microcomputer interfaced to the vertical autoprofiler (VAP) described earlier (Chung et al. 1981. J. Lipid Res. 22: 10...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1982-08-01 00:00:00
abstract::A positive effect of all-trans retinoic acid (ATRA) on white adipose tissue (WAT) oxidative and thermogenic capacity has been described and linked to an in vivo fat-lowering effect of ATRA in mice. However, little is known about the effects of ATRA on mitochondria in white fat. Our objective has been to characterize t...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:10.1194/jlr.M053652
更新日期:2015-06-01 00:00:00
abstract::Studies on plasma triglycerides (TG) were performed in 10 nonobese and 16 obese patients with hypothyroidism and in 13 with hyperthyroidism. Nonobese, hypothyroid patients generally had normal levels of TG, but obese patients often had hypertriglyceridemia. In most hypothyroid patients 1-thyroxine treatment lowered pl...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1981-02-01 00:00:00
abstract::Animal studies have suggested that angiopoietin-like 4 (Angptl4) regulates adiposity through central and peripheral mechanisms. The aim of this study was to investigate whether serum concentration and adipose tissue expression of Angptl4 are associated with obesity-related parameters in humans. Altogether, 75 dizygoti...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:10.1194/jlr.P015867
更新日期:2011-08-01 00:00:00
abstract::An improved preparation of proteolipid from bovine brain white matter is described. The product obtained by repeated acetone precipitation is completely soluble in chloroform-methanol and has a fairly constant composition: 35% protein, 40% galactocerebroside, and about 25% phospholipid. ...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1967-01-01 00:00:00
abstract::Pre-packed reverse phase columns (Bond Elut) were used for the separation of complex lipids, such as phosphatidylcholine, cerebrosides, sulfatides, and gangliosides, from their respective water-soluble radioactive precursors after their in vitro biosynthesis. After an incubation in vitro, the entire reaction mixture i...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1985-01-01 00:00:00
abstract::N-3 polyunsaturated fatty acids from marine oil have been shown to decrease T cell-mediated immune function both in animals and humans, and to inhibit the mitogen-induced lymphoproliferative response when added to lymphocyte culture medium. As phosphatidic acid (PA) is a key mediator of the mitogenic process, the pres...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1998-04-01 00:00:00
abstract::ATP-binding cassette transporter A1 (ABCA1) plays a crucial role in apoA-I lipidation, a key step in reverse cholesterol transport. cAMP induces apoA-I binding activity and promotes cellular cholesterol efflux. We investigated the role of the cAMP/protein kinase A (PKA) dependent pathway in the regulation of cellular ...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:10.1194/jlr.m200235-jlr200
更新日期:2002-12-01 00:00:00
abstract::The effects of fish oil supplementation (14.5 g n-3 fatty acids/day) on plasma lipoprotein particles in healthy volunteers were assessed by high resolution 13C and 1H nuclear magnetic resonance (NMR) spectroscopy. Resonances not previously observed in the 13C and 1H spectra of plasma and isolated lipoproteins were det...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1996-08-01 00:00:00
abstract::The acylation of glycerol-3-phosphate by acyl-CoA is regarded as the first committed step for the synthesis of the lipoidal moiety in glycerolipids. The direct acylation of glycerol in mammalian tissues has not been demonstrated. In this study, lipid biosynthesis in myoblasts and hepatocytes was reassessed by conducti...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:2001-12-01 00:00:00