China
Africa
Explore chapters and articles related to this topic
Neonatal adrenoleukodystrophy/disorders of peroxisomal biogenesis
Published in William L. Nyhan, Georg F. Hoffmann, Aida I. Al-Aqeel, Bruce A. Barshop, Atlas of Inherited Metabolic Diseases, 2020
William L. Nyhan, Georg F. Hoffmann, Aida I. Al-Aqeel, Bruce A. Barshop
Chemical analysis of the lipid of the brain revealed an increase in cholesterol esters and a diminution in constituents of myelin [1]. Hexacosanoic (C26:0) acid accounted for 25 percent of the total fatty acid [24, 41]. Examination of the VLCFA of the plasma and cultured fibroblasts also reveals accumulation of VLCFA. Levels are similar to those found in X-linked ALD [4]. The mean C26:C22 ratio in fibroblasts in two patients [3] was 0.5, while that in ALD was 0.7. The value for controls was 0.03. The accumulation tends to be less than that seen in Zellweger syndrome. In another patient, the ratio was 1.8 [9]. The levels of C26:0 in postmortem liver and adrenal were higher than those reported in ALD [3]. Accumulation of VLCFA has also been observed in retina [23]. Oxidation of lignoceric acid (C24:0) in cultured fibroblasts is impaired [3], and the level of activity is similar to that of cells derived from patients with ALD. Defective plasmalogen synthesis tends to be less than that of Zellweger syndrome. A systematic approach to the biochemical diagnosis of peroxisomal disorders has been set out [42]. Biochemical tests are supplemented with functional studies in cultured fibroblasts, and by molecular analysis. It is clear that peroxisomal fission disorders may be elucidated in patients with normal levels of peroxisomal metabolites. Complementation studies may be used to determine which of many PEX genes is abnormal.
Structures and Properties of Self-Assembled Phospholipids in Excess Water
Published in E. Nigel Harris, Thomas Exner, Graham R. V. Hughes, Ronald A. Asherson, Phospholipid-Binding Antibodies, 2020
The second major group of phospholipids is sphingophospholipid which contains sphin-gosine as the backbone instead of glycerol. As shown in Figure 1, sphingosine is a long chain amino alcohol with a primary hydroxy 1 on carbon 1, and amino group on carbon 2, a secondary hydroxy 1 on carbon 3, and a trans double bond between carbons 4 and 5. The stereo configuration about the asymmetric carbons 2 and 3 is d-erythro. This compound is thus called trans-D-erythro-1,3-dihydroxy-2-amino-4-octadecene. The most commonly found sphingophospholipid, particular in the myelin sheath of nerve cells, is sphingomyelin (SPM) or 2-ALacylsphingosyl-l-phosphocholine (Figure 2), in which a phosphorylcholine is esteri-fied to the primary hydroxyl group of the sphingosine base at carbon 1 position and a fatty acid is amide-linked to the nitrogen of the sphingosine base at carbon 2 position. Typical fatty acids linked via the amide bond are stearic [C(18)], behenic [C(22)], lignoceric [C(24)] and nervonic [C(24:1A15)]. The sphingosine base contributes its linear hydrophobic chain with a fixed length of 15 carbons as one of the two chains in the region of the hydrophobic tail. Since this hydrocarbon chain is significantly shorter than that of lignoceric acid, one of the most abundant fatty acids found in natural sphingolipids, natural sphingomyelin molecules thus exhibit a marked hydrocarbon chain length asymmetry (Figure 4).
Lipids of Histoplasma Capsulatum
Published in Rajendra Prasad, Mahmoud A. Ghannoum, Lipids of Pathogenic Fungi, 2017
Each of the compounds was analyzed for fatty acid, long-chain base and inositol. The results are summarized in Table 2. All of the compounds yielded one mol of long-chain base which was identified as phytosphingosine. Additionally, compounds II and III also possessed a small amount of DL-erythrodihydrosphingosine. The thin layer chromatography solvent system of benzene:chloroform:acetic acid (90:10:1, v/v) separates the methyl esters of nonhydroxylated fatty acids from mono-and dihydroxy fatty acids. When this was employed with the methyl esters of the Histoplasma compounds, compound II exhibited only non-hydroxy fatty acids whereas only monohydroxy fatty acid methyl esters were evident in the methanolysates of the other four compounds. Each compound exhibited one mol of fatty acid/mol of phosphorous and one mol of inositol/mol of phosphorous (Table 2). The fatty acid moiety of all of the compounds was lignoceric acid (24:0). Compound II had a nonhydroxlyated 24:0 fatty acid in the ceramide moiety whereas the others contained mostly hydroxylated 24:0.
Omega-3 fatty acid decreases oleic acid by decreasing SCD-1 expression in the liver and kidney of a cyclosporine-induced nephropathy rat model
Published in Renal Failure, 2019
Su Mi Lee, Mi Hwa Lee, Young Ki Son, Seong Eun Kim, Yongsoon Park, Seo Hee Rha, Won Suk An
The erythrocyte membrane contents of SFA, stearic acid, and lignoceric acid contents were observed to be significantly decreased, whereas myristic and palmitic acid contents were significantly increased in the CsA-treated group when compared to the control group (Table 2). However, omega-3 FA supplementation reversed the total SFA content and markedly increased palmitic acid content. Omega-6 and arachidonic acid (AA) contents were lower in the omega-3 FA-supplemented CsA group than in the CsA group. Omega-3 index (EPA and DHA contents) is a new risk factor for death from coronary heart disease [22]. The EPA, DHA, and omega-3 index significantly increased in the omega-3 FA supplemented CsA group when compared to the CsA group. Additionally, the ratio of AA to EPA and omega-6 to omega-3 also significantly decreased in the omega-3 FA-supplemented CsA group when compared to the CsA group. Finally, the oleic acid content in erythrocyte membrane significantly increased in the CsA group when compared to the control group, which was significantly decreased by omega-3 FA supplementation.
Erythrocyte-derived liposomes for the treatment of inflammatory diseases
Published in Journal of Drug Targeting, 2022
A. Olival, S. F. Vieira, V. M. F. Gonçalves, C. Cunha, M. E. Tiritan, A. Carvalho, R. L. Reis, H. Ferreira, N. M. Neves
Some fatty acids, such as DHA and EPA, present several health benefits [22–24]. Therefore, the profile and content of different fatty acids in the lipidic extracts and EDLs were determined by GC-MS. An example of the GC-MS chromatograms obtained for the EDLs samples is presented in Supplemental Figure 1. The saturated fatty acid stearic acid and the unsaturated fatty acids EPA and DHA were successfully identified and quantified (Table 1). The higher fatty acids concentration obtained in EDLs is related to the use of concentrated extracts in their production. Other fatty acids, including palmitic acid, oleic acid, linoleic acid, arachidonic acid and lignoceric acid, as well as cholesterol esters were also identified (Supplemental Figure 1).