Abstract:
:A subgroup of peroxisomal disorders, peroxisome biogenesis defects (PBD), can be differentiated by elevated levels of C(27) bile acids in plasma and bile. Patients with peroxisomal disorders, who lack the ability to chain-shorten the C(27) bile acid intermediates into C(24) bile acids, show elevated levels of C(27) bile acids, notably 3 alpha,7 alpha-dihydroxy-5 beta-cholest-26-oic acid and 3 alpha,7 alpha,12 alpha-trihydroxy-5 beta-cholestan-26-oic acid. C(27) bile acids are normally estimated against other bile acid standards, by time-consuming gas chromatography-mass spectrometry and liquid chromatography-tandem mass spectrometry methods, in plasma (minimum of 50 microl). In this article we describe the quantitation of unconjugated di- and trihydroxy C(27) bile acids in 5-microl plasma samples and 3-mm blood spots, using deuterium-labeled internal standards. The synthesis of (2)H(3)-labeled di- and trihydroxycoprostanic acids is described. The sample preparation and analysis by electrospray tandem mass spectrometry (ES-MS/MS) takes less than 1 h and features dimethylaminoethyl ester derivatives. The levels of the di- and trihydroxy bile acids are significantly higher in PBD patients than in age-matched control subjects for both plasma and blood spots collected at birth (some stored for up to 18 years). Excellent correlation is observed between the C(26:0)/C(22:0) very long chain fatty acid (VLCFA) ratio and the levels of trihydroxy C(27) bile acids in plasma from PBD patients. The ES-MS/MS method can be used to rapidly screen for PBD patients in plasma samples with elevated C(26:0)/C(22:0) VLCFA ratios and in archived collections of neonatal blood spots. - Johnson, D. W., H. J. ten Brink, R. C. Schuit, and C. Jakobs. Rapid and quantitative analysis of unconjugated C(27) bile acids in plasma and blood samples by tandem mass spectrometry. J. Lipid Res. 2001. 42: 9;-16.
journal_name
J Lipid Resjournal_title
Journal of lipid researchauthors
Johnson DW,ten Brink HJ,Schuit RC,Jakobs Ckeywords:
subject
Has Abstractpub_date
2001-01-01 00:00:00pages
9-16issue
1eissn
0022-2275issn
1539-7262journal_volume
42pub_type
杂志文章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::The effect of fatty acids on secretion of triglyceride-rich lipoprotein (d less than 1.006 g/ml) by CaCo-2 cells was studied. Of the fatty acids studied, oleic acid (18:1) was the most potent stimulator of newly synthesized triglyceride secretion in triglyceride-rich lipoproteins followed in descending order by 18:2, ...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1988-11-01 00:00:00
abstract::The skin surface represents our interface with the external environment, and as such, is our first line of defense against microbial colonization and infection. Lipids at the skin surface are thought to underlie at least part of an antimicrobial barrier. Some of these lipids are synthesized in the epidermis and are ca...
journal_title:Journal of lipid research
pub_type: 杂志文章,评审
doi:10.1194/jlr.R700016-JLR200
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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
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doi:
更新日期:1983-03-01 00:00:00
abstract::X-linked adrenoleukodystrophy (X-ALD) is a peroxisomal disorder caused by impaired degradation of very long-chain fatty acids (VLCFAs) due to mutations in the ABCD1 gene responsible for VLCFA transport into peroxisomes. Lorenzo's oil, a 4:1 mixture of glyceryl trioleate and glyceryl trierucate, has been used to reduce...
journal_title:Journal of lipid research
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doi:10.1194/jlr.M044586
更新日期:2014-03-01 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::Mitochondrial cholesterol is maintained within a narrow range to regulate steroid and oxysterol synthesis and to ensure mitochondrial function. Mitochondria acquire cholesterol through several pathways from different cellular pools. Here we have characterized mitochondrial import of endosomal cholesterol using Chinese...
journal_title:Journal of lipid research
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doi:10.1194/jlr.M029942
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abstract::A woman with primary lipoprotein lipase (LPL) deficiency developed marked hypertriglyceridemia, pancreatitis, eruptive xanthomas, and unusual palmar xanthomas during pregnancy. Hypotheses to account for the palmar xanthomas were that oxidative modification of triglyceride (TG)-rich lipoproteins occurred due to increas...
journal_title:Journal of lipid research
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abstract::none. ...
journal_title:Journal of lipid research
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abstract::PAF-dependent transacetylase (TA) modifies the functions of platelet-activating factor (PAF), a potent inflammatory lipid, either by transferring the acetyl group from PAF to lysophospholipids (TAL activity), or to sphingosine (TAS activity) or by hydrolyzing PAF (acetylhydrolase activity). In stimulated endothelial c...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:10.1194/jlr.M200292-JLR200
更新日期:2003-02-01 00:00:00
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 tr...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1997-03-01 00:00:00
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::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 accurate quantitation of high density lipoproteins has recently assumed greater importance in view of studies suggesting their negative correlation with coronary heart disease. High density lipoproteins may be estimated by measuring cholesterol in the plasma fraction of d > 1.063 g/ml. A more practical approach is...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1978-01-01 00:00:00
abstract::It has been demonstrated that the alkenylacyl class of ethanolamine phospholipid (PE) represents one of the major forms of eicosapentaenoic acid (EPA)-containing phospholipid in the circulating platelets isolated from human subjects consuming a fish oil concentrate. Since the alkenylacyl PE from human platelets is enr...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1989-01-01 00:00:00
abstract::We have investigated the extent to which modifications in the essential fatty acid content of mammalian cells can affect prostaglandin production. Swiss mouse 3T3 cells stimulated with the calcium ionophore A23187 produced 1.7 to 7 times more prostaglandin E(2) (PGE(2)) when the cultures were supplemented with linolei...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1982-05-01 00:00:00
abstract::Atherogenic mixed dyslipidemia associates with oxidative stress and defective HDL antioxidative function in metabolic syndrome (MetS). The impact of statin treatment on the capacity of HDL to inactivate LDL-derived, redox-active phospholipid hydroperoxides (PCOOHs) in MetS is indeterminate. Insulin-resistant, hypertri...
journal_title:Journal of lipid research
pub_type: 临床试验,杂志文章
doi:10.1194/jlr.P068585
更新日期:2016-11-01 00:00:00
abstract::A novel water-soluble polysaccharide fraction, CME-1, with a molecular mass of 27.6 kDa and containing mannose and galactose in a respective ratio of 4:6, was prepared from Cordyceps sinensis mycelia and identified by NMR and GC-MS. In the current study, we examined whether CME-1 has anti-inflammatory effects in RAW26...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:10.1194/jlr.M011015
更新日期:2011-03-01 00:00:00
abstract::The 3-thia fatty acids, tetradecylthioacetic acid and 3,10-dithiadicarboxylic acid are mitochondrion and peroxisome proliferators. Administration of these promotes an increased transport of endogenous fatty acids to these organelles and a higher capacity of beta-oxidation. Administration of 3-thia fatty acids to rats ...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1998-03-01 00:00:00
abstract::Endothelial lipase (EL) has been shown to be a critical determinant for high density lipoprotein cholesterol levels in vivo; therefore, assays that measure EL activity have become important for the discovery of small molecule inhibitors that specifically target EL. Here, we describe fluorescent Bodipy-labeled substrat...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:10.1194/jlr.D600041-JLR200
更新日期:2007-02-01 00:00:00
abstract::Protein farnesylation is a form of posttranslational modification that occurs in most, if not all, eukaryotic cells. Inhibitors of protein farnesyltransferase (PFTIs) have been developed as anticancer chemotherapeutic agents. Using the knowledge gained from the development of PFTIs for the treatment of cancer, researc...
journal_title:Journal of lipid research
pub_type: 杂志文章,评审
doi:10.1194/jlr.R500016-JLR200
更新日期:2006-02-01 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::EPA and DHA are not biologically equivalent; however, their individual activity on B cells is unknown. We previously reported fish oil enhanced murine B-cell activity in obesity. To distinguish between the effects of EPA and DHA, we studied the ethyl esters of EPA and DHA on murine B-cell function as a function of tim...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:10.1194/jlr.M049809
更新日期:2014-07-01 00:00:00
abstract::Various properties of retinyl ester hydrolysis in the liver and the retinal pigment epithelium (RPE) have been studied, yet the relationship between the retinyl ester hydrolase (REH) activities in these tissues of the same species is not known. In the present study, REH activities in bovine liver and RPE microsomes we...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1996-09-01 00:00:00
abstract::Remnant-like emulsions were prepared with lipid compositions similar to remnants derived from triacylglycerol-rich lipoproteins. When injected into the bloodstream of conscious mice the remnant-like emulsions labeled with cholesteryl[13C]oleate were metabolized in the liver and the appearance of 13CO2 in the breath wa...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1998-03-01 00:00:00
abstract::Cholesterol monohydrate crystal formation was studied in supersaturated model bile solutions, containing unlabeled cholesterol, sodium cholate and soybean phosphatidylcholine, and tracer amounts of [3H]cholesterol. Solutions were either seeded with cholesterol crystals to initiate growth, or not seeded to allow self-n...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1983-07-01 00:00:00
abstract::Senescence-accelerated mouse-prone (SAMP1; SAMP1@Umz) is an animal model of senile amyloidosis with apolipoprotein A-II (apoA-II) amyloid fibril (AApoAII) deposits. This study was undertaken to investigate the effects of dietary fats on AApoAII deposits in SAMP1 mice when purified diets containing 4% fat as butter, sa...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:10.1194/jlr.M200405-JLR200
更新日期:2003-04-01 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::The genetic variability of apolipoprotein E (apoE) influences plasma lipoprotein levels, and allele frequencies differ between African Americans and Caucasians. As African Americans have higher lipoprotein [a] (Lp[a]) levels than Caucasians, we investigated the effects of the apoE gene on allele-specific apolipoprotei...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:10.1194/jlr.M600431-JLR200
更新日期:2007-03-01 00:00:00
abstract::Scavenger receptor class B type I (SR-BI) and ABCA1 are structurally dissimilar cell surface proteins that play key roles in HDL metabolism. SR-BI is a receptor that binds HDL with high affinity and mediates both the selective lipid uptake of cholesteryl esters from lipid-rich HDL to cells and the efflux of unesterifi...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:10.1194/jlr.M300358-JLR200
更新日期:2004-07-01 00:00:00