Abstract：
:Abetalipoproteinemia (ABL) and chylomicron retention disease (CMRD) are extremely rare recessive forms of hypobetalipoproteinemia characterized by intestinal lipid malabsorption and severe vitamin E deficiency. Vitamin E is often supplemented in the form of fat-soluble vitamin E acetate, but fat malabsorption considerably limits correction of the deficiency. In this crossover study, we administered two different forms of vitamin E, tocofersolan (a water-soluble derivative of RRR-α-tocopherol) and α-tocopherol acetate, to three patients with ABL and four patients with CMRD. The aims of this study were to evaluate the intestinal absorption characteristics of tocofersolan versus α-tocopherol acetate by measuring the plasma concentrations of α-tocopherol over time after a single oral load and to compare efficacy by evaluating the ability of each formulation to restore vitamin E storage after 4 months of treatment. In patients with ABL, tocofersolan and α-tocopherol acetate bioavailabilities were extremely low (2.8% and 3.1%, respectively). In contrast, bioavailabilities were higher in patients with CMRD (tocofersolan, 24.7%; α-tocopherol acetate, 11.4%). Plasma concentrations of α-tocopherol at 4 months were not significantly different by formulation type in ABL or CMRD. This study provides new insights about vitamin E status in ABL and CMRD and suggests the potential of different formulations as treatment options.
journal_name
J Lipid Resjournal_title
Journal of lipid researchauthors
Cuerq C,Henin E,Restier L,Blond E,Drai J,Marçais C,Di Filippo M,Laveille C,Michalski MC,Poinsot P,Caussy C,Sassolas A,Moulin P,Reboul E,Charriere S,Levy E,Lachaux A,Peretti Ndoi
10.1194/jlr.M085043subject
Has Abstractpub_date
2018-09-01 00:00:00pages
1640-1648issue
9eissn
0022-2275issn
1539-7262pii
jlr.M085043journal_volume
59pub_type
杂志文章abstract：:The effect of fat feeding on adipocyte insulin binding was examined to expand a study of adaptive changes in plasma membrane functions. Cells from rats fed a high fat (L) diet for five to seven days bound less insulin and showed a decreased response to insulin (glucose oxidation) compared to those from rats fed a high...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1976-11-01 00:00:00
abstract：:Atherosclerosis is a chronic inflammatory response characterized by the accumulation of cells of innate and acquired immune systems within the intima of the arterial wall. Macrophages are the predominant participant in innate immune responses in atherosclerosis. Protein receptors expressed by macrophages and endotheli...
journal_title：Journal of lipid research
pub_type： 杂志文章,评审
doi：10.1194/jlr.R400015-JLR200
更新日期：2005-03-01 00:00:00
abstract：:Neu-Laxova syndrome (NLS) is a very rare autosomal recessive congenital disorder characterized by disturbed development of the central nervous system and the skin and caused by mutations in any of the three genes involved in de novo l-serine biosynthesis: PHGDH, PSAT1, and PSPH l-Serine is essential for the biosynthes...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：10.1194/jlr.P087536
更新日期：2018-12-01 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：:The ability to exogenously present cell-surface receptors in high-affinity conformations in a synthetic system offers an opportunity to provide host cells with protection from pathogenic toxins. This strategy requires improvement of the synthetic receptor binding affinity against its native counterpart, particularly w...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：10.1194/jlr.M007401
更新日期：2010-09-01 00:00:00
abstract：:The basic protein protamine strongly inhibited hydrolysis of triolein emulsified with soybean phosphatidylcholine (PC) by pancreatic and carboxylester lipases; 10 micrograms/ml protamine, about 1000 times lower than the concentration of bovine serum albumin for the same effect, inhibited triolein hydrolysis completely...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1996-07-01 00:00:00
abstract：:HDL cholesterol levels are decreased in Crohn's disease, a tumor necrosis factor-alpha (TNF-alpha)-driven chronic inflammatory condition involving the gastrointestinal tract. ATP-binding cassette transporter A1 (ABCA1), one of several liver X receptor (LXR) target genes, is a cell surface transporter that mediates the...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：10.1194/jlr.M002410
更新日期：2010-06-01 00:00:00
abstract：:Lipoproteins internalized by the LDL receptor (LDLR) are released from this receptor in endosomes through a process that involves acid-dependent conformational changes in the receptor ectodomain. How acidic pH promotes this release process is not well understood. Here, we assessed roles for six histidine residues for ...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：10.1194/jlr.M070938
更新日期：2017-02-01 00:00:00
abstract：:A method has been described for the measurement of apoB concentration and specific activity in very low density lipoprotein (VLDL) and low density lipoprotein (LDL) during metabolic studies. For measurement of specific activity, apoB was separated from other apolipoproteins and lipids by precipitation in, and subseque...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1983-09-01 00:00:00
abstract：:Polyunsaturated fatty acids, including docosahexaenoic acid (DHA, 22:6n-3), modulate immune responses and exert beneficial immunosuppressive effects, but the molecular mechanisms inhibiting T-cell activation are not yet elucidated. Lipid rafts have been shown to play an important role in the compartmentalization and m...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：10.1194/jlr.M500033-JLR200
更新日期：2005-09-01 00:00:00
abstract：:In mice, the yolk sac appears to play a crucial role in nourishing the developing embryo, especially during embryonic days (E) 7;-10. Lipoprotein synthesis and secretion may be essential for this function: embryos lacking apolipoprotein (apo) B or microsomal triglyceride transfer protein (MTP), both of which participa...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1999-11-01 00:00:00
abstract：:The LDL receptor-related protein-1 (LRP1) is highly expressed in numerous cell types, and its impairment is associated with obesity, diabetes, and fatty liver disease. However, the mechanisms linking LRP1 to metabolic disease are not completely understood. Here, we compared the metabolic phenotype of C57BL/6J wild typ...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：10.1194/jlr.RA120001141
更新日期：2020-12-09 00:00:00
abstract：:Rabbits were fed either 10% coconut oil, 10% coconut oil and 1% beta-sitosterol, 10% coconut oil and 1% cholesterol, or 10% coconut oil and 1% beta-sitosterol plus 1% cholesterol for 4 weeks. Microsomal membranes from intestines of animals fed the 1% beta-sitosterol diet had 48% less cholesterol and were enriched twof...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1983-04-01 00:00:00
abstract：:Recent reports from this laboratory indicate that exposure of cholesterol-loaded macrophages to high density lipoprotein 3 (HDL3) stimulates not only cholesterol efflux, but also results in a two- to threefold increase in apoE accumulation in the media (Dory, L., 1989. J. Lipid Res. 30: 809-816). The present experimen...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1991-05-01 00:00:00
abstract：:Niacin, the first antidyslipidemic drug, has been at the center stage of lipid research for many decades before the discovery of statins. However, to date, despite its remarkable effects on lipid profiles, the clinical outcomes of niacin treatment on cardiac events is still debated. In addition to its historically wel...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：10.1194/jlr.S092007
更新日期：2019-04-01 00:00:00
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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
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journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1967-05-01 00:00:00
abstract：:Long-chain fatty acids are the most important substrates for the heart. In addition, they have been shown to affect signalling pathways and gene expression. To explore the effects of long-chain fatty acids on cardiac gene expression, neonatal rat ventricular myocytes were cultured for 48 h with either glucose (10 mm),...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：2000-01-01 00:00:00
abstract：:Immunological tolerance is one of the fundamental aspects of the immune system. The CD4(+)CD25(+) regulatory T (Treg) cells have emerged as key players in the development of tolerance to self and foreign antigens. However, little is known about the endogenous factors and mechanisms controlling their suppressive capaci...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：10.1194/jlr.M900101-JLR200
更新日期：2009-12-01 00:00:00
abstract：:Chiral phase high performance liquid chromatographic resolution of sn-1,2(2,3)- and X-1,3-diacylglycerols generated by partial Grignard degradation from natural triacylglycerols was carried out using a chiral column (25 cm x 4.6 mm i.d.) containing (R)-(+)-1-(1-napthyl)ethylamine polymer chemically bonded to 300A wide...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1990-09-01 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
更新日期：2008-01-01 00:00:00
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journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1993-04-01 00:00:00
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journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1993-02-01 00:00:00
abstract：:HDL-associated paraoxonase type 1 (PON1) can protect LDL and HDL against oxidative modification in vitro and therefore may protect against cardiovascular disease. We investigated the effects of PON1 levels, activity, and genetic variation on high density lipoprotein-cholesterol (HDL-C) levels, circulating oxidized LDL...
journal_title：Journal of lipid research
pub_type： 临床试验,杂志文章,随机对照试验
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更新日期：2005-03-01 00:00:00
abstract：:To investigate the relative turnover of esterified polyunsaturated fatty acids in diacylglycerophospholipids and plasmalogens in isolated cardiac myocytes, we characterized the phospholipid composition and distribution of radiolabel in different phospholipid classes and in individual molecular species of diradyl choli...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1991-07-01 00:00:00
abstract：:Two novel mutations were identified in a compound heterozygous male with lecithin:cholesterol acyltransferase (LCAT) deficiency. Exon sequence determination of the LCAT gene of the proband revealed two novel heterozygous mutations in exons one (C110T) and six (C991T) that predict non-conservative amino acid substituti...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：1998-09-01 00:00:00
abstract：:The ATP binding cassette (ABC) half-transporters ABCG5 and ABCG8 facilitate biliary and intestinal removal of neutral sterols. Here, we identify a binding site for the orphan nuclear receptor liver receptor homolog-1 (LRH-1) at nt 134-142 of the ABCG5/ABCG8 intergenic region necessary for the activity of both the ABCG...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：10.1194/jlr.C400002-JLR200
更新日期：2004-07-01 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：:Synthesis of apolipoprotein (apo)E in hepatocytes leads to both secretion and retention in cell surface pools. Inclusion of Brefeldin A to HepG2 cells prompted a rapid decrease of cell surface apoE to about 37% of control values after a 3-h incubation. The t(1/2) for this dynamic pool was estimated to be 15 min. In co...
journal_title：Journal of lipid research
pub_type： 杂志文章
doi：
更新日期：2001-09-01 00:00:00
abstract：:Five samples from three different types of 1500-year-old Mediterranean amphorae, as well as from a contemporary oil lamp found in the same deposit, were analyzed for the presence of lipid residues. Each sample of finely ground amphorae powder weighed 1-2 g. The abundance of interfering secondary products makes thin-la...
journal_title：Journal of lipid research
pub_type： 历史文章,杂志文章
doi：
更新日期：1981-07-01 00:00:00
