Abstract:
:The time course of the clearance from the blood and the tissue localization of [14C]L-glucosylceramide, a nonmetabolizable enantiomorph of D-glucosylceramide that accumulates in Gaucher's disease, has been determined. 14C-labeled L-glucosylceramide injected intravenously in the form of micelles or liposomes is rapidly removed from the circulation. Most of this lipid is taken up by the liver where it is found in both hepatocytes and nonparenchymal cells. This sphingolipid analog is promptly cleared from hepatocytes and a significant portion is recovered in the bile. The clearance of [14C]L-glucosylceramide from Kupffer cells is greatly prolonged in comparison with its brief residence in hepatocytes. These findings have significant implications regarding the pathogenesis and treatment of Gaucher's disease.
journal_name
J Lipid Resjournal_title
Journal of lipid researchauthors
Tokoro T,Gal AE,Gallo LL,Brady ROsubject
Has Abstractpub_date
1987-08-01 00:00:00pages
968-72issue
8eissn
0022-2275issn
1539-7262journal_volume
28pub_type
杂志文章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::Unesterified cholesterol is an essential structural component of the plasma membrane of every cell. During evolution, this membrane came to play an additional, highly specialized role in the central nervous system (CNS) as the major architectural component of compact myelin. As a consequence, in the human the mean con...
journal_title:Journal of lipid research
pub_type: 杂志文章,评审
doi:10.1194/jlr.R400004-JLR200
更新日期:2004-08-01 00:00:00
abstract::The technique of freeze fracture was used to visualize triglyceride (TG) hydrolysis and the production of lipolytic products (LPs) in vitro and in vivo in the presence of bile salts (BS). Three systems were investigated: pure lipolytic products (oleic acid and monoolein) in the presence of a pure bile salt (taurodeoxy...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1986-08-01 00:00:00
abstract::Lysosomal acid lipase (LAL) is essential for the hydrolysis of triglycerides (TG) and cholesteryl esters (CE) in lysosomes. A mouse model created by gene targeting produces no LAL mRNA, protein, or enzyme activity. The lal-/- mice appear normal at birth, survive into adulthood, and are fertile. Massive storage of TG a...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:2001-04-01 00:00:00
abstract::Lipoprotein (a) [Lp(a)] is a highly atherogenic lipid particle. Although earlier reports suggested that Lp(a) levels are mostly determined by genetic factors, several recent studies have revealed that Lp(a) induction is also caused by chronic inflammation. Therefore, we aimed to examine whether cytokine blockade by mo...
journal_title:Journal of lipid research
pub_type: 临床试验,杂志文章
doi:10.1194/jlr.P052209
更新日期:2015-05-01 00:00:00
abstract::A sensitive and reproducible method has been developed for separation of the major serum lipoproteins from 1 ml or less of human serum by isopycnic density gradient ultracentrifugation. The serum, applied to a step gradient (total volume 12.8 ml), was spun for 48 hr at 38,000 rpm at 10 degrees C and, in each of the fr...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1977-11-01 00:00:00
abstract::Phospholipase was isolated (in two fractions) from Vipera palestinae venom and it was shown to possess phospholipase A activity (hydrolyzing diacyl-sn-glycerophosphorylcholines, e.g., lecithin, in the 2-position) as well as lysophospholipase (phospholipase B) activity (hydrolyzing 1-monoacyl-sn-glycerophosphorylcholin...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1973-05-01 00:00:00
abstract::Distinct isoforms of long-chain acyl-CoA synthetases (ACSLs) may partition fatty acids toward specific metabolic cellular pathways. For each of the five members of the rat ACSL family, we analyzed tissue mRNA distributions, and we correlated the mRNA, protein, and activity of ACSL1 and ACSL4 after fasting and refeedin...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:10.1194/jlr.M600150-JLR200
更新日期:2006-09-01 00:00:00
abstract::14C-labeled oleic acid and (3)H-labeled monoether in a bile salt solution were perfused through three types of plastic tubing. Large proportions of lipid were lost to the walls of silicone rubber and polyvinyl chloride tubes. The major portion of the lipid lost was recoverable only when chloroform-methanol was perfuse...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1971-09-01 00:00:00
abstract::To consider possible interaction of the phospholipid membrane with calcium ions, crystal structures of calcium dl-alpha- and beta-glycerophosphates (alpha- and beta-CaGs, respectively) were investigated by X-ray diffraction methods. After many attempts, relatively large single crystals of beta-CaG were prepared from t...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1992-07-01 00:00:00
abstract::In skin fibroblasts grown from four children with a homozygous form of type II hyperlipoproteinemia, the feedback control of sterol synthesis and the inhibitory effect on hydroxymethylglutaryl (HMG) CoA reductase activity by serum or low density lipoprotein were present, though diminished compared with the effects in ...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1975-03-01 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::Fatty acid compositions of glycerophosphatides of developing chick embryonic brain and liver were compared. In brain, ethanolamine and serine glycerophosphatides contained 30-40% polyunsaturated fatty acids, lecithin almost none (except for arachidonic). In the liver, these acids were equally distributed in the phosph...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1966-09-01 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::This paper describes the purification and some of the properties of an enzyme from human spleen that catalyzes the hydrolysis of sphingomyelin with the formation of ceramide and phosphoryl choline. The enzyme, which is located in the subcellular particulate fraction that sediments between 700 and 8500 g, is readily ma...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1967-05-01 00:00:00
abstract::Livers from fed or 24-hr fasted male rats were perfused in a recycling system. VLDL labeled with [1-14C]oleate (95% in triglyceride), produced in separate perfusions of livers from fed rats, was added to the medium as a pulse. Uptake of VLDL 14C-labeled triglyceride by livers from fasted rats was less than that from f...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1987-04-01 00:00:00
abstract::Mouse plasma from strains C57BL/6J and C3H/HeJ includes a high density lipoprotein (HDL) fraction containing apolipoprotein A-I which migrates in the prebeta region upon agarose gel electrophoresis, similar to the prebeta HDL previously reported in humans. This prebeta A-I lipoprotein species has a buoyant density of ...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1990-02-01 00:00:00
abstract::We used a mouse C57BL/6J×CASA/Rk intercross to map a locus on chromosome 14 that displayed a gender-dependent effect on cholesterol absorption from the intestine. Studies in congenic animals revealed a complex locus with multiple operating genetic determinants resulting in alternating gender-dependent phenotypic effec...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:10.1194/jlr.M034579
更新日期:2013-03-01 00:00:00
abstract::A novel metabolite of d-delta-tocopherol was isolated from the urine of rats given d-3,4-[3H2]-delta-tocopherol intravenously. The metabolite was collected from the urine of rats given d-delta-tocopherol in the same manner as that of the labeled compound. It was found that the metabolites consisted of sulfate conjugat...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1984-01-01 00:00:00
abstract::Using the "ghost method" previously applied to study the binding of palmitate to bovine serum albumin (BSA) (Bojesen, I. N., and E. Bojesen. 1992. J. Lipid Res. 33: 1327-1334) we have measured the water-phase concentrations of arachidonate (AR) and oleate (OL) in 165 mM KCl and in 165 mM NaCl, 2 mM phosphate buffer at...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1994-05-01 00:00:00
abstract::The hepatic synthesis of sterol was measured in the male Mongolian gerbil (Meriones unguiculatus) in vivo following the administration of [3H]water by monitoring the incorporation of radioactivity into digitonin-precipitable sterol. A diurnal rhythm in cholesterol synthesis was exhibited under conditions of ad libitum...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1981-07-01 00:00:00
abstract::Boiled coffee contains an unidentified lipid that raises serum cholesterol. We studied the effects of the ingestion of coffee oil fractions of increasing purity in volunteers in order to identify the cholesterol-raising factor. In 15 volunteers who ingested 0.75 g/d of a non-triglyceride-fraction from coffee oil for 4...
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:1994-04-01 00:00:00
abstract::The tear film covers the anterior eye and the precise balance of its various constituting components is critical for maintaining ocular health. The composition of the tear film amphiphilic lipid sublayer, in particular, has largely remained a matter of contention due to the limiting concentrations of these lipid amphi...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:10.1194/jlr.M044826
更新日期:2014-02-01 00:00:00
abstract::Our previous studies have shown accumulation of GM2 ganglioside during ethanol-induced neurodegeneration in the developing brain, and GM2 elevation has also been reported in other brain injuries and neurodegenerative diseases. Using GM2/GD2 synthase KO mice lacking GM2/GD2 and downstream gangliosides, the current stud...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:10.1194/jlr.M056580
更新日期:2015-08-01 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::There is growing evidence of the capacity of vitamin A to regulate the expression of the genetic region that encodes apolipoproteins (apo) A-I, C-III, and A-IV. This region in turn has been proposed to modulate the expression of hyperlipidemia in the commonest genetic form of dyslipidemia, familial combined hyperlipid...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1999-03-01 00:00:00
abstract::We have developed a simplified and improved high performance liquid chromatography (HPLC) method for the detection and quantitation of tissue lipid using a new laser light-scattering detector (Varex model ELSD II). This detector has a limit of sensitivity of 50 ng for neutral lipid and 200 ng for most phospholipids wi...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1990-11-01 00:00:00
abstract::Dihydroxyacetone phosphate acyl transferase (DHAP-AT), alkyl dihydroxyacetone phosphate synthase (alkyl-DHAP-synthase), and glycerol-3-phosphate acyltransferase (GPAT) activities were investigated under optimal assay conditions using highly purified organelle preparations. The data presented clearly indicate that GPAT...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1993-03-01 00:00:00
abstract::The enzymic incorporation of choline-1,2-(14)C from CDP-choline-1,2-(14)C into phosphatidylcholine by spinach leaf preparations was characterized. The enzyme catalyzing the incorporation, choline phosphotransferase, had a pH optimum of about 8.0 and required either Mn(2+) or Mg(2+) as cofactor. The saturation concentr...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1971-07-01 00:00:00
abstract::Our previous studies have demonstrated the activity of oncostatin M (OM) in stimulating the transcription of the human LDL receptor (LDLR) gene in HepG2 cells through a sterol-independent regulatory mechanism. The current studies were designed to determine whether this in vitro property of OM could be recapitulated in...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:10.1194/jlr.M400425-JLR200
更新日期:2005-06-01 00:00:00