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Showing 1-30 of 37 results for "52200" within Papers
Tang-Chia Chung et al.
Analytica chimica acta, 611(1), 113-118 (2008-02-27)
Docosanoic (C22), tetracosanoic (C24) and hexacosanoic (C26) acids are saturated very-long-chain fatty acids (VLCFA) present at trace levels in biosamples. VLCFA can be used as potential biomarkers for the diagnosis of hereditary diseases such as X-linked adrenoleukodystrophy. Because the analytes
Aya Okahashi et al.
Clinica chimica acta; international journal of clinical chemistry, 401(1-2), 141-143 (2009-01-13)
Fatty acid metabolism has a close relationship with metabolic syndrome. Saturated very long-chain fatty acid (hexacosanoic acid; C26:0) was recently reported to be associated with cardiovascular risk factors in adults. Eighty-eight children (47 male, 41 female; average age, 10.9 y)
Ana C Matias et al.
Free radical biology & medicine, 43(10), 1458-1465 (2007-10-16)
Changes in plasma membrane permeability caused by H2O2 were recently found to be involved in the adaptation to H2O2, but the mechanism responsible for this change remains largely unknown. Here this mechanism was addressed and two lines of evidence showed
Biju K Pillai et al.
The Journal of biological chemistry, 284(48), 33296-33304 (2009-10-06)
Abnormalities in the transport of saturated very long chain fatty acids (VLCFA; >C18:0) contribute to their toxic levels in peroxisomal disorders of fatty acid metabolism, such as adrenoleukodystrophy and adrenomyeloneuropathy. We previously showed that VLCFA desorb much slower than normal
Pedro Brites et al.
Brain : a journal of neurology, 132(Pt 2), 482-492 (2008-11-22)
Peroxisomes are organelles responsible for multiple metabolic pathways including, the biosynthesis of plasmalogens, a class of phospholipids, and the beta-oxidation of very-long-chain fatty acids (VLCFA). Lack of peroxisomes or dysfunction in any of their normal functions is the cellular basis
Alastair Wilkins et al.
Multiple sclerosis (Houndmills, Basingstoke, England), 15(12), 1525-1527 (2009-12-08)
In clinical practice it is not uncommon for patients with adrenomyeloneuropathy or female carriers of adrenoleucodystrophy to have a presenting history and examination compatible with multiple sclerosis. This suggests that there may be an under-diagnosis of adrenoleucodystrophy and its variants
P Aubourg et al.
The New England journal of medicine, 329(11), 745-752 (1993-09-09)
Adrenomyeloneuropathy is an X-linked recessive disorder characterized by myelopathy, peripheral neuropathy, and cerebral demyelination, which develop in association with the accumulation of very-long-chain fatty acids. The administration of oleic and erucic acids inhibits the synthesis of very-long-chain fatty acids. Recently
Wen-jie Qin et al.
Zhong yao cai = Zhongyaocai = Journal of Chinese medicinal materials, 30(8), 959-961 (2007-12-14)
To study the chemical constituents of Heterosmilax yunianensis. The compounds were isolated and repeatedly purified with chromatograph and the structures were elucidated by physico-chemical properties and spectral analysis. Eight compounds were obtained and elucidated as beta-sitosterol (I), glycerol monopalmitate (II)
Gursev S Dhaunsi et al.
Cell biochemistry and function, 23(1), 65-68 (2004-11-27)
Very long chain fatty acids (VLCFAs) are exclusively oxidized in peroxisomes and their levels are significantly increased in tissues of patients with peroxisomal disorders. Although the biochemical indicators of peroxisomal dysfunction, such as elevated VLCFAs, are well known, the mechanisms
Laura Bannach Jardim et al.
Brain & development, 32(3), 180-190 (2009-03-10)
X-linked adenoleukodystrophy is a genetic disease that affects the degradation of very long-chain fatty acids. In male patients, common pictures are the cerebral form (CALD), myeloneuropathy (AMN), and Addison-only. To describe the clinical course of affected male patients from South
H W Moser et al.
Pediatric research, 16(3), 172-175 (1982-03-01)
Amniocentesis was performed in two women heterozygous for adrenoleukodystrophy (ALD). One fetus was male, and the hexacosanoic acid (C26) level in the cultured amniotic cells was 0.808 microgram per mg of protein, compared to 0.104 +/- 0.069 (S.D.) in controls.
Natalia Yu Ryabova et al.
European biophysics journal : EBJ, 39(8), 1167-1176 (2009-12-17)
The outermost epidermal layer, the stratum corneum (SC), is the main skin barrier. Studies of SC model systems enable characterization of the influence of individual lipids on the organization of the SC lipid matrix, which is the main pathway of
Marion Deon et al.
Metabolic brain disease, 23(1), 43-49 (2007-11-21)
X-linked adrenoleukodystrophy (X-ALD) is an inherited disorder of peroxisomal metabolism, biochemically characterized by deficient beta-oxidation of saturated very long chain fatty acids (VLCFA). The consequent accumulation of these fatty acids in different tissues and in biological fluids is associated with
Jun Cheng et al.
Zhongguo Zhong yao za zhi = Zhongguo zhongyao zazhi = China journal of Chinese materia medica, 28(12), 1153-1155 (2004-12-25)
To investigate the constituents from the Stems of Spatholobus suberectus. The chemical constituents were isolated by column chromatography and their strutures were identified on base of physical and spectral data. Five compounds were isolated and identified as 2-methoxy-4-(2'-hydroxyethyl)-phenyl-1-O-beta-D-glucopyranoside (1), n-butyl-O-beta-D-fructo
Zahid Muneer et al.
PloS one, 9(9), e108655-e108655 (2014-09-26)
The inherited peroxisomal disorder X-linked adrenoleukodystrophy (X-ALD), associated with neurodegeneration and inflammatory cerebral demyelination, is caused by mutations in the ABCD1 gene encoding the peroxisomal ATP-binding cassette (ABC) transporter ABCD1 (ALDP). ABCD1 transports CoA-esters of very long-chain fatty acids (VLCFA)
Guan-yuan Chen et al.
Clinica chimica acta; international journal of clinical chemistry, 438, 126-134 (2014-08-26)
As fatty acids play an important role in biological regulation, the profiling of fatty acid expression has been used to discover various disease markers and to understand disease mechanisms. This study developed an effective and accurate comparative fatty acid analysis
M Aasim Khan et al.
Natural product research, 24(7), 610-620 (2010-04-20)
Phytochemical investigation of the fruit peels of Citrus reticulata Blanco (Rutaceae) resulted in the isolation of three new phytoconstituents along with n-hexacosonoic acid. Their structures have been established as 18betaH-urs-5,11-dien-3beta-ol-11-one-3beta-D-glucopyranosyl-(4'-->1'')-D-glucopyranosyl-6''-(3''',4'''-dihydroxyl)-benzoate (reticulataursenoside), stigmast-5-en-3beta-ol-3beta-D-glucopyranosyl-4'-eicosanoate (citrusteryl arachidate), and lanost-5-en-3beta-ol-3beta-D-glucopyranosyl-4'-eicosanoate (citruslanosteroside) on the basis
P A Watkins et al.
The Journal of clinical investigation, 83(3), 771-777 (1989-03-01)
Peroxisomal function was evaluated in a male infant with clinical features of neonatal adrenoleukodystrophy. Very long chain fatty acid levels were elevated in both plasma and fibroblasts, and beta-oxidation of very long chain fatty acids in cultured fibroblasts was significantly
Nicol Kruska et al.
Biochimica et biophysica acta, 1852(5), 925-936 (2015-01-15)
X-linked adrenoleukodystrophy (X-ALD) is a severe neurodegenerative disorder resulting from defective ABCD1 transport protein. ABCD1 mediates peroxisomal uptake of free very-long-chain fatty acids (VLCFA) as well as their CoA-esters. Consequently, VLCFA accumulate in patients' plasma and tissues, which is considered
Stéphane Fourcade et al.
Human molecular genetics, 17(12), 1762-1773 (2008-03-18)
X-linked adrenoleukodystrophy (X-ALD) is a fatal neurodegenerative disorder, characterized by progressive cerebral demyelination cerebral childhood adrenoleukodystrophy (CCALD) or spinal cord neurodegeneration (adrenomyeloneuropathy, AMN), adrenal insufficiency and accumulation of very long-chain fatty acids (VLCFA) in tissues. The disease is caused by
Hugo W Moser et al.
Archives of neurology, 62(7), 1073-1080 (2005-07-13)
To identify asymptomatic boys with X-linked adrenoleukodystrophy who have a normal magnetic resonance image (MRI), and to assess the effect of 4:1 glyceryl trioleate-glyceryl trierucate (Lorenzo's oil) on disease progression. Eighty-nine boys (mean +/- SD baseline age, 4.7 +/- 4.1
A Sadeghi-Nejad et al.
The New England journal of medicine, 322(1), 13-16 (1990-01-04)
Adrenoleukodystrophy, a sex-linked peroxisomal disorder that results in the impaired oxidation of long-chain saturated fatty acids and causes neurologic impairment, is a rare cause of Addison's disease in children. Adrenomyeloneuropathy is the name given to a biochemically identical but milder
Marion Deon et al.
Journal of the neurological sciences, 247(2), 157-164 (2006-06-06)
X-linked adrenoleukodystrophy (X-ALD) is a peroxisomal disorder biochemically characterized by the accumulation of very long chain fatty acids (VLCFA), particularly hexacosanoic acid (C(26:0)) and tetracosanoic acid (C(24:0)), in tissues and biological fluids. Although patients affected by this disorder predominantly present
Walter C Hubbard et al.
Molecular genetics and metabolism, 89(1-2), 185-187 (2006-07-11)
Utilizing combined liquid chromatography-tandem mass spectrometry (LC-MS/MS) as the analytical method, we have demonstrated a ten to sixtyfold excess of lysophosphatidyl choline containing hexacosanoic acid (26:0) in dried blood spots on a filter paper matrix from 25 male patients with
Wei-Yi Hsu et al.
Analytical chemistry, 82(16), 6814-6820 (2010-08-14)
Peroxisomal disorders are characterized biochemically by elevated levels of very long chain fatty acids (VLCFAs) in serum. Herein, we describe a novel approach for quantification of VLCFAs in serum, namely, eicosanoic acid (C20:0), docosanoic acid (C22:0), tetracosanoic acid (C24:0), and
Oswald Quehenberger et al.
Journal of lipid research, 51(11), 3299-3305 (2010-07-31)
The focus of the present study was to define the human plasma lipidome and to establish novel analytical methodologies to quantify the large spectrum of plasma lipids. Partial lipid analysis is now a regular part of every patient's blood test
M Cappa et al.
Journal of endocrinological investigation, 34(10), 753-756 (2011-03-15)
X-linked adrenoleukodystrophy/adrenomieloneuropathy (ALD/AMN) is a progressive neurodegenerative disorder due to mutations in the ABCD1 gene encoding the ABC transporter ALDP. Mutations in ALDP impair peroxisomal β-oxidation of very long chain fatty acids (VLCFA), resulting in elevated levels of VLCFA in
Marc Engelen et al.
Journal of inherited metabolic disease, 35(6), 1137-1145 (2012-03-27)
X-linked adrenoleukodystrophy (X-ALD) is caused by mutations in the ABCD1 gene encoding ALDP, an ATP-binding-cassette (ABC) transporter located in the peroxisomal membrane. ALDP deficiency results in impaired peroxisomal β-oxidation and the subsequent accumulation of very long-chain fatty acids (VLCFA; >
Wen Tang et al.
Biomarkers : biochemical indicators of exposure, response, and susceptibility to chemicals, 19(6), 505-508 (2014-08-01)
To investigate phospholipids (PLs) biomarkers in predicting outcome of patients undergoing peritoneal dialysis (PD). Twenty PD patients were followed using baseline plasma PLs with an improved online two-dimensional liquid chromatography-quadrupole time-of-flight mass spectrometry system. Significant differences were observed in eight
Yanjing Bai et al.
Journal of natural medicines, 65(3-4), 417-423 (2011-02-09)
Not only neuronal death but also neuritic atrophy and synaptic loss underlie the pathogenesis of Alzheimer's disease as direct causes of the memory deficit. Extracts of Siberian ginseng (the rhizome of Eleutherococcus senticosus) were shown to have protective effects on
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