Abstract:
:Analyses have been made of glycosphingolipids from visceral organs and brain of a patient with an unusual lipid storage disorder diagnosed initially as classical Tay-Sachs disease. Levels of the lipids from fresh-frozen sections of gray and white matter, kidney, spleen, liver, and heart from this patient were compared with those of normal juvenile controls, and the fatty acid composition of accumulated glycosphingolipids was compared with reference compounds. This patient was found to have abnormally high concentrations of a globoside in liver, kidney, and spleen, asialo G(M2) ganglioside in brain and liver, and G(M2) ganglioside in the brain. On the basis of these findings along with the clinical manifestations of Tay-Sachs disease with visceral involvement (hepatosplenomegaly) and demonstration of total deficiency of both A and B components of beta-N-acetylhexosaminidase activity, this glycosphingolipidosis is the same as two previously reported cases of G(M2) gangliosidosis with globoside accumulation and total beta-N-acetylhexosaminidase deficiency.
journal_name
J Lipid Resjournal_title
Journal of lipid researchauthors
Snyder PD Jr,Krivit W,Sweeley CCsubject
Has Abstractpub_date
1972-01-01 00:00:00pages
128-36issue
1eissn
0022-2275issn
1539-7262journal_volume
13pub_type
杂志文章abstract::Despite a complete lack of microsomal triglyceride transfer protein (MTP), L35 rat hepatoma cells secrete triglyceride-containing lipoproteins, albeit at a rate 25% of that of parental FAO hepatoma cells, which express high levels of MTP. The inability to express MTP was associated with a complete block in the secreti...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:2002-05-01 00:00:00
abstract::Rhizobium chitolipooligosaccharides (CLOSs) are heterogeneous fatty acylated N-acetyl glucosamine oligomers with variations in both the polar (hydrophilic) oligosaccharide head group and the non-polar (hydrophobic) fatty acyl chain. They trigger root hair deformation and cortical cell divisions in legume roots during ...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1997-06-01 00:00:00
abstract::Sterol-regulatory element binding protein-1c (SREBP-1c) is a transcription factor that controls lipogenesis in the liver. Hepatic SREBP-1c is nutritionally regulated, and its sustained activation causes hepatic steatosis and insulin resistance. Although regulation of SREBP-1c is known to occur at the transcriptional l...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:10.1194/jlr.M004234
更新日期:2010-07-01 00:00:00
abstract::The concentration of cholesterol carried in low density lipoproteins (LDL-C) is primarily determined by the rate at which LDL-C is produced (Jt) and the rate at which the liver takes up this particle through receptor-dependent transport (Jm). The accumulation of specific dietary fatty acids in the liver profoundly alt...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1994-09-01 00:00:00
abstract::The content of serum gangliosides was examined in VM and C57BL/6J (B6) mice that contained subcutaneous metastatic (VM) and non-metastatic (CT-2A) brain tumors, respectively. Gas-liquid chromatography (GLC) and high performance thin-layer chromatography (HPTLC) were used to analyze the serum gangliosides. N-glycolylne...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1994-01-01 00:00:00
abstract::A method for apolipoprotein (apo) E genotyping was developed using the polymerase chain reaction (PCR) with allele-specific oligonucleotide primers (ASP). Synthetic oligonucleotides with base-pair mismatches at the 3' terminus were used as primers to amplify the apoE gene in subjects previously phenotyped using isoele...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1991-01-01 00:00:00
abstract::Animal experiments show that the kidney contributes to apolipoprotein (apo)A-I catabolism. We tested relationships of HDL cholesterol (HDL-C) and apo-I with kidney function in subjects without severe chronic kidney disease. Included was a random sample of the general population (part of the PREVEND cohort). Kidney fun...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:10.1194/jlr.M005348
更新日期:2010-07-01 00:00:00
abstract::Tetradecylthioacetic acid (TTA), which cannot be beta-oxidized, exerts growth-limiting properties in glioma cells. In order to investigate the importance of modulated lipid metabolism and alterations in mitochondrial properties in this cell death process, we incubated glioma cells both with TTA and the oxidizable fatt...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:10.1194/jlr.m200312-jlr200
更新日期:2003-01-01 00:00:00
abstract::A two-step polymerase chain reaction (PCR) method for the rapid detection of the apolipoprotein B(Arg3500----Gln) mutation in a mixture of pooled blood samples is described. In the first step PCR, a short gene fragment surrounding codon 3500 is amplified. Subsequently the reaction product is subjected to a second ampl...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1992-10-01 00:00:00
abstract::Conserved lysines and arginines within amino acids 140-150 of apolipoprotein (apo) E are crucial for the interaction between apoE and the low density lipoprotein receptor (LDLR). To explore the roles of amphipathic alpha-helix and basic residue organization in the binding process, we performed site-directed mutagenesi...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:2000-07-01 00:00:00
abstract::The rate of peroxidation of unconjugated bilirubin (UCB), catalyzed by horseradish peroxidase (HRP), has been employed by Jacobsen (1969. FEBS Lett. 5: 112-114) to assess the fraction of unbound UCB in the presence of serum albumin. We used this method to examine the interactions of UCB with taurocholate (TC) at pH 8....
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1987-06-01 00:00:00
abstract::Oxidized forms of cholesterol (oxysterols) are present in atherosclerotic lesions and may play an active role in lesion development. For example, 7-ketocholesterol (7KC) inhibits cholesterol efflux from macrophage foam cells induced by apolipoprotein A-I (apoA-I). Such oxysterols may promote foam cell formation in ath...
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 aim of this study was first to examine the relationships between adiponectin gene (Apm1) polymorphisms and anthropometric indices as well as plasma adiponectin and lipoprotein/lipid levels, and then to investigate whether the presence of visceral obesity or insulin resistance may modulate the impact of these polym...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:10.1194/jlr.M400135-JLR200
更新日期:2005-02-01 00:00:00
abstract::The resistin gene is expressed in adipocytes and encodes a protein proposed to link obesity and type 2 diabetes. Increased plasma FFA is associated with insulin resistance. We examined the effect of separate FFAs on the expression of resistin mRNA in cultured murine 3T3-L1 adipocytes. The FFAs tested did not increase ...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:10.1194/jlr.M400348-JLR200
更新日期:2005-01-01 00:00:00
abstract::Subnormal HDL-cholesterol (HDL-C) and apolipoprotein (apo)AI levels are characteristic of familial hypercholesterolemia (FH), reflecting perturbed intravascular metabolism with compositional anomalies in HDL particles, including apoE enrichment. Does LDL-apheresis, which reduces HDL-cholesterol, apoAI, and apoE by ads...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:10.1194/jlr.P016816
更新日期:2011-12-01 00:00:00
abstract::Phosphorus-31 nuclear magnetic resonance (31P NMR) spectroscopy has been used to study accumulation of N-acyl-ethanolamine phospholipids in rat brains during post-decapitative ischemia. Lipids were extracted from rat brain homogenates and the extracts were thoroughly washed with aq. potassium ethylenediaminetetraaceti...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1999-03-01 00:00:00
abstract::Percent cholesterol absorption was measured in 94 normal subjects aged 17- 80 years while consuming diets generally low in cholesterol (mean intake = 226 +/- 126 mg/day). A new dual stable isotope method was used where a cholesterol tracer containing 6 extra mass units was given intravenously and another tracer with 5...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1999-02-01 00:00:00
abstract::We have previously demonstrated the potential usefulness of capillary columns coated with a high temperature polarizable phenylmethylsilicone liquid phase in plasma lipid profiling (Kuksis, A., J.J. Myher, and P. Sandra. 1990. J. Chromatogr. 500: 427-441). The present study reports improved operating conditions along ...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1993-06-01 00:00:00
abstract::Mice bearing the Ehrlich ascites tumor were fed diets rich in either coconut oil or sunflower oil. From 20 to 40% less lipid was present in the ascites tumor fluid when the mice were fed the sunflower oil diet. This was associated with a reduction in the amount of very low density lipoproteins (VLDL) and high density ...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1978-05-01 00:00:00
abstract::In an effort to explore the feasibility of converting a lipase into an esterase by modifying the lid region, we designed and characterized two novel Rhizopus chinensis lipase variants by lid swapping. The substrate specificity of an R. chinensis lipase was successfully modified toward water-soluble substrates, that is...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:10.1194/jlr.M043950
更新日期:2014-06-01 00:00:00
abstract::Solid-phase extraction (SPE) methods are easy, rapid, and reliable. Their growing popularity is in part due to their operational simplicity and cost reduction in solvents, and partly because they are easier to automate. Sphingolipids are implicated in various cellular events such as growth, differentiation, and apopto...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:2000-09-01 00:00:00
abstract::We designed a rapid method for determining mRNA content of cholesterol biosynthesis enzymes and LDL receptor (LDLR) using a ribonuclease protection assay (RPA). 32P-labeled cRNA fragments for genes of human LDLR and the enzymes HMG-CoA synthase (HMGS), HMG-CoA reductase (HMGR), mevalonate kinase (MK), farnesyl pyropho...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1995-09-01 00:00:00
abstract::Alzheimer's disease (AD) is characterized by an extensive accumulation of amyloid-β (Aβ) peptide, which triggers a set of deleterious processes, including synaptic dysfunction, inflammation, and neuronal injury, leading to neuronal loss and cognitive impairment. A large body of evidence supports that nuclear receptor ...
journal_title:Journal of lipid research
pub_type: 杂志文章,评审
doi:10.1194/jlr.R075556
更新日期:2017-10-01 00:00:00
abstract::Liver microsomal lipid peroxidation has been observed in fatal human CCl(4) poisoning, in rats with fatty livers induced by CCl(4) or by yellow phosphorus, and in mice poisoned with 1,1,2,2-tetrachloroethane. These observations suggest the possibility that other instances of toxic liver injury may involve lipid peroxi...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1971-11-01 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::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::The Niemann-Pick C1 (NPC1) protein regulates cholesterol transport from late endosomes-lysosomes to other intracellular compartments. In this article, cholesterol transport to caveolin-1 and caveolin-2 containing compartments, such as the trans-Golgi network (TGN) and plasma membrane caveolae, was examined in normal (...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:2002-04-01 00:00:00
abstract::A simplified method for the preparation of the Dittmer-Lester spray for the detection of phospholipids on thin-layer chromatograms is described. ...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1968-05-01 00:00:00
abstract::Inflammatory lipid mediators derived from arachidonic acid (AA) and docosahexaenoic acid (DHA) modify the pathophysiology of brain ischemia. The goal of this work was to investigate the formation of eicosanoids and docosanoids generated from AA and DHA, respectively, during no-flow cerebral ischemia. Rats were subject...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:10.1194/jlr.M800200-JLR200
更新日期:2008-09-01 00:00:00
abstract::Mitochondrial sterol 27-hydroxylase (EC 1.14.13.15) is an important enzyme, not only in the formation of bile acids from cholesterol intermediates in the liver but also in the removal of cholesterol by side chain hydroxylation in extrahepatic tissues. The enzyme has been assayed by complicated methods using radiolabel...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:10.1194/jlr.D200045-JLR200
更新日期:2003-12-01 00:00:00