Abstract:
:In humans, peroxisomes harbor a complex set of enzymes acting on various lipophilic carboxylic acids, organized in two basic pathways, alpha-oxidation and beta-oxidation; the latter pathway can also handle omega-oxidized compounds. Some oxidation products are crucial to human health (primary bile acids and polyunsaturated FAs), whereas other substrates have to be degraded in order to avoid neuropathology at a later age (very long-chain FAs and xenobiotic phytanic acid and pristanic acid). Whereas total absence of peroxisomes is lethal, single peroxisomal protein deficiencies can present with a mild or severe phenotype and are more informative to understand the pathogenic factors. The currently known single protein deficiencies equal about one-fourth of the number of proteins involved in peroxisomal FA metabolism. The biochemical properties of these proteins are highlighted, followed by an overview of the known diseases.
journal_name
J Lipid Resjournal_title
Journal of lipid researchauthors
Van Veldhoven PPdoi
10.1194/jlr.R005959subject
Has Abstractpub_date
2010-10-01 00:00:00pages
2863-95issue
10eissn
0022-2275issn
1539-7262pii
jlr.R005959journal_volume
51pub_type
杂志文章,评审abstract::The aim of this study was to investigate the extent to which inflammation is linked with plasma endothelial lipase (EL) concentrations among healthy sedentary men. Plasma C-reactive protein (CRP) concentrations were measured with a highly sensitive commercial immunoassay, plasma interleukin-6 (IL-6) concentrations wer...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:10.1194/jlr.P600002-JLR200
更新日期:2006-12-01 00:00:00
abstract::Monolayers of Caco-2 cells, a human enterocyte cell line, were incubated with [1-14C]15-hydroxyeicosatetraenoic acid (15-HETE), a lipid mediator of inflammation, and [1-14C]arachidonic acid. Both fatty acids were taken up readily and metabolized by Caco-2 cells. [1-14C]Arachidonic acid was directly esterified in cellu...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1990-05-01 00:00:00
abstract::Circulating triacylglycerol (TG) arises mainly from dietary fat. However, little is known about the entry of dietary fat into the major TG pool, very low-density lipoprotein (VLDL) TG. We used a novel method to study the specific incorporation of dietary fatty acids into postprandial VLDL TG in humans. Eight healthy v...
journal_title:Journal of lipid research
pub_type: 临床试验,杂志文章
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更新日期:2003-11-01 00:00:00
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journal_title:Journal of lipid research
pub_type: 杂志文章
doi:10.1194/jlr.M059089
更新日期:2015-08-01 00:00:00
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journal_title:Journal of lipid research
pub_type: 杂志文章
doi:10.1194/jlr.M031427
更新日期:2013-01-01 00:00:00
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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::Autosomal dominant Stargardt-like macular dystrophy (STGD3) in humans results from mutations in elongation of very long chain FAs-like 4 (ELOVL4), which leads to vision loss in young adults. ELOVL4 is an integral endoplasmic reticulum (ER) protein that mediates the elongation of very long chain (VLC) FAs. Mutations in...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:10.1194/jlr.M045443
更新日期:2014-04-01 00:00:00
abstract::In order to study the metabolic responses of humans consuming a diet moderately high in cholesterol content, we carried out a long-term sterol balance study, up to 25 weeks in duration. Two subjects, one normocholesterolemic and one hypercholesterolemic, were given, in sequence, a very low cholesterol diet and then a ...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1980-11-01 00:00:00
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journal_title:Journal of lipid research
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更新日期:2019-08-01 00:00:00
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journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1994-11-01 00:00:00
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journal_title:Journal of lipid research
pub_type: 杂志文章
doi:10.1194/jlr.M600525-JLR200
更新日期:2007-08-01 00:00:00
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journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1972-01-01 00:00:00
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journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1986-09-01 00:00:00
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journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1988-03-01 00:00:00
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journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1975-07-01 00:00:00
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journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1990-12-01 00:00:00
abstract::The isolated perfused rat liver was used to study the 300-800 A electron-opaque bodies which had previously been described in the liver cell Golgi apparatus, smooth endoplasmic reticulum, and space of Disse. When the perfusion medium was enriched with linoleate, the number and electron opacity of these particles incre...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1967-09-01 00:00:00
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journal_title:Journal of lipid research
pub_type: 杂志文章
doi:10.1194/jlr.M500203-JLR200
更新日期:2006-02-01 00:00:00
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journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1995-12-01 00:00:00
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journal_title:Journal of lipid research
pub_type: 杂志文章
doi:10.1194/jlr.D119000318
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journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
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pub_type: 杂志文章
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journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1981-11-01 00:00:00
abstract::Activation of cultured hepatic stellate cells correlated with an enhanced expression of proteins involved in uptake and storage of fatty acids (FA translocase CD36, Acyl-CoA synthetase 2) and retinol (cellular retinol binding protein type I, CRBP-I; lecithin:retinol acyltransferases, LRAT). The increased expression of...
journal_title:Journal of lipid research
pub_type: 杂志文章
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更新日期:2003-02-01 00:00:00
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journal_title:Journal of lipid research
pub_type: 杂志文章
doi:10.1194/jlr.M041061
更新日期:2013-10-01 00:00:00
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journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1987-11-01 00:00:00
abstract::Increased plasma concentrations of apolipoprotein A-IV (apoA-IV) in chronic renal disease suggest a metabolic role of the kidney for this antiatherogenic protein. Therefore, we investigated patients with various forms of proteinuria and found increased serum concentrations of apoA-IV in 124 nephrotic patients compared...
journal_title:Journal of lipid research
pub_type: 杂志文章
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更新日期:2006-09-01 00:00:00
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journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1999-02-01 00:00:00
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journal_title:Journal of lipid research
pub_type: 杂志文章
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更新日期:2008-09-01 00:00:00
abstract::Enzymes associated with glycerolipid biosynthesis were examined in microsomal fractions of liver and adipose tissue obtained from swine of various ages. Generally, liver glycerophosphate acyltransferase, phosphatidate phosphohydrolase, diglyceride acyltransferase, and choline phosphotransferase activities were substan...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
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