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1.
Abstract— Cholesterol esters were isolated from cerebral cortex and white matter of patients with Schilder's disease, GM1-gangliosidosis and Tay-Sachs disease, and the fatty acid composition was determined by gas-liquid chromatography. The fatty acid composition was similar among the three pathological conditions, but it was entirely different from that reported for cholesterol esters of normal brain. Lecithin and ethanolamine phospholipids were isolated from the same brain specimens, treated with snake venom phospholipase A, and the fatty acids at the a’and β-positions of the glycerol moiety were determined separately. The fatty acid composition of cholesterol esters was similar to that of the β-position fatty acids of lecithin of white matter in all samples, and was quite different from those of the a'-position of lecithin, or of the a’or β-position of ethanolamine phospholipids. The results indicate that the source of fatty acids for cholesterol esterification in nonspecific sudanophilic demyelination is different from that in normal brain, and that the most likely source is the β-linked fatty acids of lecithin. There are two possible enzymic mechanisms; activation of phospholipase A and subsequent esterification of the liberated β-position fatty acids to cholesterol, or direct transacylation by lecithin-cholesterol acyl transferase.  相似文献   

2.
FATTY ACID ABNORMALITY IN ADRENOLEUKODYSTROPHY   总被引:20,自引:10,他引:10  
—Recent clinical and morphological evidence established that adrenoleukodystrophy is a distinct X-linked genetic disorder. Fatty acid compositions of lipids in the brain, adrenal and serum from seven patients were examined. Cholesterol esters of both brain and adrenal contained substantial proportions of fatty acids longer than C22 (11.8–41.9% of total in the brain and 13.4-34.8% of total in the adrenal), while cholesterol esters from normal and pathological control specimens contained very little. These very long chain fatty acids were generally saturated in brain cholesterol esters but significant amounts of unsaturated long chain fatty acids were also present in adrenal cholesterol esters. The long chain fatty acids showed bell-shaped distribution with C25 or C26 at the peak. Ganglio-sides from patients’white matter also showed increased proportions of very long-chain fatty acids, up to 50% of the total. Qualitatively similar but much milder fatty acid abnormalities were also found in galactosylceramide of the brain. On the other hand, fatty acids and fatty aldehydes of brain glycerophospholipids, adrenal free fatty acids, triglycerides and glycerophospholipids were not abnormal. Furthermore, serum cholesterol esters from two patients did not show the long-chain fatty acid abnormality found in brain and adrenal cholesterol esters. Sequential extractions with acetone and hexane established that the characteristic birefringent material in the brain and adrenal is indeed cholesterol esters with very long chain fatty acids. This type of fatty acid abnormality has not been described in other pathological conditions and may well represent the unique biochemical abnormality that is directly related to the fundamental genetic defect underlying adrenoleukodystrophy.  相似文献   

3.
Abstract— The variation with age of the fatty acid composition of the major lipids in human brain myelin was compared with that of cerebral white matter from the same region. The myelin was isolated from the semiovale centre of the cerebrum of 27 subjects neonatal to old aged. The phospholipid, cholesterol and galactolipid concentrations were determined in all the samples, as were the proportions of the major phospholipid classes. The proportions of cholesterol and especially of the galactolipids increased in myelin during the first 6 months, and in cerebral white matter up to 2 years. During this period the individual phospholipids also varied substantially. Serine phosphoglycerides and especially sphingomyelins increased, and choline phosphoglycerides decreased. The fatty acid patterns of ethanolamine phosphoglycerides (EPG) and sphingomyelins underwent the largest changes. The proportions of saturated fatty acids in EPG diminished rapidly, and there was an increase of monoenoic acids. Fatty acids of the linoleic acid series showed a peak between 4 and 12 months, after which time their proportion slowly diminished to old age. The major fatty acid of this series was docosatetraenoic acid, 22:4 (n-6), which constituted more than 25% of total fatty acids at the maximum level. The fatty acid changes were larger in cerebral white matter, but from 2 years of age the EPG fatty acid pattern in myelin was similar to that in white matter. The fatty acid changes in serine and choline phosphoglycerides of myelin with maturation were much less striking than in EPG but of a similar type. In myelin sphingomyelin the proportion of saturated long-chain fatty acids, C16-C22, diminished, while that of monoenoic acids increased and continued to do so up to old age. From 2 years of age the fatty acid patterns in myelin and cerebral white matter were quite similar. Also the fatty acid patterns of cerebrosides and sulphatides in cerebral white matter and myelin were the same except for the first 2 months of life. The same fatty acid changes occurred in cerebrosides and sulphatides as in the sphingomyelins, i.e. increased proportions of unsaturated (monoenoic) acids. The proportions of 24:1 and 24h:1 and of the odd-numbered fatty acids 25:1 and 23h:1 continued to increase to old age. The variations of the individual lipid fatty acid patterns were small except in the youngest age classes, in which the variations were presumably ascribable to the difficulty in determining the gestational age.  相似文献   

4.
CHANGES IN CEREBRAL CORTICAL LIPIDS IN COBALT-INDUCED EPILEPSY   总被引:2,自引:0,他引:2  
Abstract– In control rats and in rats rendered epileptic by insertion of cobalt slivers into the cerebral cortex, total free fatty acids, free cholesterol, esterified cholesterol, triglycerides and phospholipids were measured in normal and lesion areas of cerebral cortex. The cortical lipid profile of the adult rat resembled that of the whole brain of very young rats rather than that of adult whole brain, with the principal differences from whole adult brain being lower total lipid content, increased proportions of phosphatidyl choline in the phospholipid fraction, and higher levels of cholesterol esters. Cobalt-induced epilepsy was associated with significant changes in cerebral cortical lipids in the area of the lesion and in the non-necrotic tissue adjacent to the lesion. The total lipid in the area of the lesion decreased sharply as a result of reductions in free cholesterol and total phospholipids. The levels of cholesterol esters and triglycerides increased in the area of the lesion, and cholesterol esters were also increased in the adjacent tissue. In addition there were decreases in the proportion of phosphatidyl ethanolamine in the phospholipids from the lesion site and adjacent tissue and decreases in the proportions of oleic, arachidonic and nervonic acids (unsaturated acids), and an increase in the proportions of lignoceric acid in the phospholipids. In the site of the lesion only, we observed a decrease in phospholipid palmitic acid and an appreciable increase in the proportions of an unidentified long-chained fatty acid.  相似文献   

5.
Selectivity factors (Vm/Km) for human and rat lecithin: cholesterol acyltransferases (LCAT) for the transfer of various acyl groups from the 2-position of phosphatidylcholine were determined. By multiplying these values by the proportions of acyl groups at the 2-position of phosphatidylcholine, one can predict the proportions of molecular species of cholesterol ester which will be synthesized by LCAT. In human subjects fasted overnight, the molecular composition of plasma cholesterol ester was found to reflect the LCAT selectivity relatively accurately. This result supports the concepts that hepatic acyl-CoA:cholesterol acyltransferase (ACAT) does not contribute significantly to the synthesis of plasma cholesterol ester and that removal of cholesterol ester from plasma is not selective with respect to molecular species under these conditions. In contrast to the results with humans, the molecular composition of plasma cholesterol ester formed in spontaneously hypertensive rats fed a high-cholesterol diet and then fasted overnight differs from that which is predicted from LCAT selectivity and the proportion of various fatty acids at the 2-position of phosphatidylcholine: these results suggest that cholesterol ester is formed mainly via the ACAT reaction.  相似文献   

6.
Abstract— Glial cells were isolated from bovine white matter by differential centrifugation. The fatty aldehyde and fatty acid compositions of ethanolamine glycerophosphatides (EGP), serine glycerophosphatides (SGP) and choline glycerophosphatides (CGP) were determined by gas-liquid chromatography. The fatty acid compositions of the sphingo-lipids including sphingomyelin, cerebroside and cerebroside sulphate, and of minor lipid components including cholesterol esters and triglycerides, were also determined by gas-liquid chromatography. The relative proportions correlated closely with the results obtained by O'B rien and S ampson (1965 b ) for adult human brain. The fatty aldehyde compositions of the glycerophosphatides were more closely related to the corresponding fatty acid compositions of the plasma membrane than of the mitochondria. Long-chain fatty acids (19–26 carbon atoms) were detected in sphingomyelin, cerebroside and cerebroside sulphate; this indicates that chain-elongation beyond C18 occurs in the glial cells.  相似文献   

7.
Abstract The changes of fatty acids in larvae of the wheat blossom midge, Sitodiplosis mosellana (Gehin) at different periods were examined by gas choromatography. There were 10–16 kinds of fatty acids, of which the predominant ingredients were palmitic (C16:0), oleic (C18:1) and linoleic (C18:2) acids which were more than 95% in total fatty acids, stearic acid (C18:0) about 2%‐3.5% and any of the others was less than 1%. The fatty acid compositions increased from mid‐May, when larvae of the wheat blossom midge left the wheat‐ears and fallen on the ground, to April of next year before pupating and emerging. No arachidic acid (C20.0) was discovered in over‐summering, over‐wintering as well as inactive over‐wintered larvae. The content of saturated fatty acids in over‐summering, overwintering as well as inactive over‐wintered larvae were less than those of in active over‐wintered larvae and wheat‐ear larvae. Therefore, changes of the arachidic acid and the proportions of saturated fatty acids/unsaturated fatty acids could be used as one of the biochemical criteria to determine the active state and the degree of diapause in larvae of the wheat blossom midge.  相似文献   

8.
We report on the presence of arachidonic acid in larval and adult tissues of the primary screwworm, Cochliomyia hominivorax and of the secondary screwworm, C. macellaria. Arachidonic acid is present in the phospholipids of whole animal extracts of both species. This fatty acid appears to be accumulated during the larval stages, because proportions of arachidonic acid were higher in adults than in larvae. These insects probably obtain the arachidonic acid from dietary phospholipids. We also report on a phospholipase A2 activity in midgut preparations from third instars of the primary screwworm. Phospholipase A2 is responsible for hydrolyzing fatty acids from the sn-2 position of dietary phospholipids to release essential fatty acids. The screwworm enzyme is similar to mammalian digestive phospholipase A2s because it depends on calcium for high catalytic activity, it is sensitive to the site-specific inhibitor oleyloxyethylphosphorylcholine, and it interacts with heparin. We further characterized the screwworm midgut phospholipase A2 by altering the reaction conditions, including reaction time, radioactive substrate concentration, protein concentration, pH and temperature. We speculate that the biological significance of this enzyme relates to acquiring essential fatty acids, including arachidonic acid, from dietary phospholipids.  相似文献   

9.
Abstract— The contents and the fatty acid composition of cholesterol esters were analysed in developing rat brain. The total content did not exceed 20 μg/brain throughout development. Elimination of serum by adequate perfusion was essential for accurate results. Two separate events appeared to affect the levels of cholesterol esters in developing rat brain, one probably reflecting general developmental changes and the other apparently related to myelination. On either a unit weight or a whole brain basis, the curves appeared to be a superimposition of the two events. There was an underlying developmental change, which was characterized on a unit weight basis by the highest level of cholesterol esters immediately after birth and a steady decline to the adult level by 30 days of age or which on the basis of whole brain was characterized by a steady increase throughout the development. A period of transient increase was superimposed on this underlying developmental change between the ages of 7 and 27 days and corresponded to the period of active myelination. The major fatty acids of rat brain cholesterol esters were palmitic, palmitoleic, oleic and arachidonic acids. Palmitic and palmitoleic acids decreased in proportion while oleic acid increased, as the animal matured. The fatty acid composition of serum cholesterol esters was distinctly different from that of brain cholesterol esters; those from serum contained much higher proportions of linoleic and arachidonic acids and much less palmitoleic and oleic acids.  相似文献   

10.
The dried-fruit moth was reared on three diets differing in quantity and types of fatty acids. Triacylglycerols (TAG) were the major lipid present in both larvae and adults and their fatty acid compositions reflected dietary fatty acids to some extent. However, larval and adult contained relatively high levels of 16:0, 16:1, and 18:1 even when these acids were not predominant in the dietary lipid. Diacylglycerols were present in larvae and adults at lower levels than TAG and their fatty acid compositions were similar to those of the corresponding TAG.Adults contained more phospholipid (PL) and cholesterol than larvae regardless of diet employed. Phosphatidyl-ethanolamine and phosphatidyl-choline were the major PL present with appreciable levels of diphosphatidyl-glycerol and lesser amounts of lysophosphatidyl-ethanolamine and choline, sphingomyelin, phosphatidyl-serine, and inositol being present. The higher level of PL noted in the adults were due to a general increase in all of the individual phospholipids and not to any specific PL. Dietary fatty acids did influence fatty acid compositions of larval and adult PL. However, there appeared to be compensations in the proportions of other fatty acids in the PL in that high 18:1 was accompanied by lowered levels of 18:2 and high 18:2 was accompanied by lowered levels of saturated fatty acids.In every case adult PL contained more polyenoic fatty acids than did larval PL. There were no apparent compensations in other fatty acids accompanying these changes.  相似文献   

11.
The fatty acid spectra of 6 periphyton communities developed in laboratory streams at different combinations of light intensity and current velocity were determined by gas-liquid chromatography and silver nitrate thin-layer chromatography. Differences in species composition of the communities apparently had no striking effect on proportions of palmitic and stearic acids, whereas concentrations of myristic, palmitoleic, oleic, linoleic, and linolenic acids and a C20:5 acid were more closely related to taxonomic differences. In general, communities dominated by blue-green algae exhibited relatively high proportions of oleic, linolenic, and linolenic acids and low proportions of palmitoleic acid and a C20:5 acid, as compared to communities consisting primarily of diatoms. The data also indicated an inverse relationship between fatty acid redundancy and species diversity.  相似文献   

12.
Studies were carried out on the lipid composition of a halotolerant Staphylococcus epidermidis isolated in pure culture from a growth medium for extreme halophiles containing 25% NaCl. The four major polar lipid components in this bacterium were found to be: (a) glycerophosphoryl diglucosyl diglyceride (10% by weight) with structure 3(1)-O-(-sn-glycerol-1-phosphoryl-6'-O=(beta-D glucopyranosyl-(1 leads to 6)- O-beta-D-glucopyranosyl)-1(3),2-diacyl-sn-glycerol; (b) diglucosyl diglyceride (15% by weight) with structure 3(1)-O-(beta-D-glucopyranosyl (1 leads to 6)-O-beta-D-glucopyranosyl)-1(3),2-diacyl-sn-glycerol; (c) monoglucosyl diglyceride (3% by weight) with structure 3(1)-O-(beta-D-glucopyranosyl)-1(3),2-diacyl-sn-glycerol, and (d) phosphatidylglycerol (60% by weight) with structure 1,2 diacyl-sn-glycero-3-phosphoryl-1'-sn-glycerol. Phosphatidic acid, cardiolipin, lysophosphatidylglycerol and three unidentified phospholipids were also detected in small amounts. Each lipid component had essentially the same fatty acid composition namely, anteiso-15:0 (60-75%), anteiso-17:0 (18-24%), iso-17:0 (8--10%), and small amounts of palmitic and stearic acids (2-5%). The fatty acids were non-randomly distributed in phosphatidylglycerol, the shorter chain anteiso 15:0 fatty acid being exclusively esterified to the 2-position and the longer chain anteiso- and iso-17:0 fatty acids at the 1-position. The fatty acid composition was not affected by increaseing NaCl content in the medium in the rande 0--15% but the proportion of anteiso-15:0 increased greatly when the salt concentration was increased to 25%. The proportions of ionic polar lipids were modified to give an increased net negative charge per mol ionic lipids when NaCl in the medium was increased from 15 to 25%, but the proportions of neutral glycolipids remained fairly constant.  相似文献   

13.
LIPID COMPOSITION OF OPTIC NERVE MYELIN   总被引:1,自引:0,他引:1  
Abstract— Myelin was isolated from bovine optic nerves by differential ultracentrifugation and its lipid composition was analysed. Optic nerve myelin contained 76·3 per cent lipid. The major lipids were cholesterol, ethanolamine glycerophosphatides (EGP) and cerebroside. Serine glycerophosphatides (SGP), sphingomyelin and cerebroside sulphate were present in smaller proportions. EGP and SGP contained 34·6 and 0·5 per cent aldehydes. The major fatty aldehydes were palmitaldehyde, stearaldehyde and octadecenaldehyde. The fatty acids of EGP, SGP and choline glycerophosphatides (CGP) were chiefly 16:0, 18:0 and 18:1, with small proportions of 20 and 22 carbon polyunsaturates. The sphingolipids contained predominantly saturated and monounsaturated fatty acids of chain lengths of 20–26 carbon atoms. Optic nerve myelin and white matter myelin resembled one another closely in overall lipid composition and in the fatty acid compositions of their constituent lipids. Optic nerve myelin and white matter myelin are chemically similar membranes, but both of these differ in their lipid composition from spinal root myelin.  相似文献   

14.
Cholesterol esterification in rabbit plasma   总被引:1,自引:1,他引:0       下载免费PDF全文
1. When [4-(14)C]cholesterol, attached to beta-globulin or dispersed with Tween 20, was incubated with fresh rabbit (New Zealand albino females) plasma, 30-47% esterification was observed. The optimum pH was 6.8. This esterification was accomplished by the transfer of fatty acids from the C-2 position of lecithin (phosphatidylcholine) to cholesterol. 2. There was no evidence that triglycerides or free fatty acids participated directly in this reaction. Lecithins with labelled palmitic acid, oleic acid and linoleic acid in the 2-position yielded 3.2, 4.8 and 6.8% of cholesteryl esters respectively. This pattern reflects that which is normally observed in the cholesteryl esters of rabbit plasma and supports the concept that plasma cholesteryl esters originate from the plasma. 3. Snake venom (containing phospholipase A), sulphoevernan [an alpha-(1-->3,1-->4)-sulphopolyglucan with 12% sulphur], thiol-blocking agents (p-chloromercuribenzoate and N-ethylmaleimide), or an atherogenic diet (stock diet supplemented with 1% cholesterol for 8 weeks) were all effective inhibitors of this cholesterol esterification.  相似文献   

15.

Background

Patients with phenylketonuria (PKU) have to follow a lifelong phenylalanine restricted diet. This type of diet markedly reduces the intake of saturated and unsaturated fatty acids especially long chain polyunsaturated fatty acids (LC-PUFA). Long-chain saturated fatty acids are substrates of mitochondrial fatty acid oxidation for acetyl-CoA production. LC-PUFA are discussed to affect inflammatory and haemostaseological processes in health and disease. The influence of the long term PKU diet on fatty acid metabolism with a special focus on platelet eicosanoid metabolism has been investigated in the study presented here.

Methodology/Principal Findings

12 children with PKU under good metabolic control and 8 healthy controls were included. Activated fatty acids (acylcarnitines C6–C18) in dried blood and the cholesterol metabolism in serum were analyzed by liquid chromatographic tandem mass spectrometry (LC-MS/MS). Fatty acid composition of plasma glycerophospholipids was determined by gas chromatography. LC-PUFA metabolites were analyzed in supernatants by LC-MS/MS before and after platelet activation and aggregation using a standardized protocol. Patients with PKU had significantly lower free carnitine and lower activated fatty acids in dried blood compared to controls. Phytosterols as marker of cholesterol (re-) absorption were not influenced by the dietary fatty acid restriction. Fatty acid composition in glycerophospholipids was comparable to that of healthy controls. However, patients with PKU showed significantly increased concentrations of y-linolenic acid (C18:3n-6) a precursor of arachidonic acid. In the PKU patients significantly higher platelet counts were observed. After activation with collagen platelet aggregation and thromboxane B2 and thromboxane B3 release did not differ from that of healthy controls.

Conclusion/Significance

Long-term dietary fatty acid restriction influenced the intermediates of mitochondrial beta-oxidation. No functional influence on unsaturated fatty acid metabolism and platelet aggregation in patients with PKU was detected.  相似文献   

16.
Chromatographically homogeneous phosphatidylcholine identified by thin-layer chromatography on silica gel G was isolated from mycelia of Aspergillus oryzae or egg yolk by a combination of alumina and silicic acid column chromatography. Although the 2-position in both Asp. oryzae- and egg yolk-phosphatidylcholine was occupied nearly exclusively by unsaturated fatty acids, significant proportions of unsaturated fatty acids were also found in the 1 -position of Asp. oryzae-phosphatidylcholine. In nitrogen gas-sparging anaerobic culture of Saccharomvces sake Kyokai No. 7, supplementing the basal synthetic medium with phosphatidylcholine enhanced the yeast growth and fermentative activity, whereas adding ergosteryl oleate enhanced alcohol-endurability. Supplementation with both phosphatidylcholine and ergosteryl oleate promoted the yeast growth, fermentative activity and alcohol-endurability of cells.  相似文献   

17.
The main glycerolipids (monogalactosyl-, digalactosyl-, sulphoquinovosyl diacylglycerol, phosphatidylglycerol) from five blue-green algae (Microcystis, Anabaena, Nostoc, Oscillatoria, Tolypothrix) were analyzed for fatty acid composition, occurrence of diglyceride species and positional distribution of fatty acids between thesn-1- andsn-2-position of glycerol. In contrast to eucaryotic plants biosynthetically closely related lipids (monogalactosyl-, digalactosyl-, trigalactosyl diacylglycerol) show nearly identical diglyceride moieties, whereas sulphoquinovosyl diacylglycerol and phosphatidylglycerol are separated from galactolipids by composition as well as occurrence of fatty acids. On the other hand the positional distribution of fatty acids in all lipids is controlled exclusively by chain length and not by degree of unsaturation with C18-fatty acids at thesn-1- and C16-fatty acids at thesn-2-position. These results show that in procaryotic organisms the diversity in diglyceride portions of lipids is reduced as compared to eucaryotic organisms, but nevertheless does exist.Abbreviations MGD, DGD, TGD, SQD monogalactosyl-, digalactosyl-, trigalactosyl-, sulphoquinovosyl diacylglycerol - PG phosphatidyl glycerol  相似文献   

18.
1. The lipid composition of serum and liver and some properties of serum lecithin: cholesterol acyltransferase of the horse were investigated. 2. Phospholipids and cholesterol were the major components of serum lipids and the concentration of triglyceride was considerably low. The concentration of liver lipids was comparable with that of other mammals. 3. Fatty acid composition of serum cholesterol ester resembled that of the 2-position of lecithin, except palmitic acid. 4. The activity of serum cholesterol esterifying enzyme was found to be 0.03-0.09 mumol/hr per ml. There was an equimolar decrease in free cholesterol and lecithin during incubation, and changes in unsaturated fatty acids in these two components were in good agreement. 5. Cholesterol esterification was reversibly inhibited by 5,5'-dithiobis-(2-nitrobenzoic acid). The acyl-transferase had a specificity for linoleic acid.  相似文献   

19.
—Adult rats were killed 16 h, 48 h, 6 days and 21 days after intracerebral application of n-[15,16-3H]tetracosanoic acid (lignoceric acid). After incorporation into complex lipids with a strong preference for the ester-bound fatty acids of glycerophospholipids, radioactivity decreased with time. The incorporated activity into the amide-bound fatty acids of sphingolipids was also shown to decrease, with exception of the cerebroside of the hydroxy fatty acid type (cerebron fraction). Only negligible amounts of labelled triglyceride and cholesterol ester could be detected. The fatty acids derived from the complex lipids were analysed by radio gas chromatography. It was revealed that some of the applied labelled lignoceric acid was hydroxylated and incorporated into the cerebron fraction while the rest had their chains shortened. In the latter case all even and odd numbered chain lengths down to C18 and C16 (stearic and palmitic acid) were detected. At this stage, the pool of the degradation products of lignoceric acid is stabilized by the preferred incorporation of fatty acids of these chain lengths into glycerophospholipids. A time-dependent desaturation to oleic acid from stearic acid was observed.  相似文献   

20.
Abstract— By chromatography on borate-coated silicic acid, glucocerebrosides, galactocerebrosides, sulfatides and sphingomyelins from brain tissue could be efficiently separated. Adult rat brain was found to contain 54.1 ± 1.5 nmol of glucocerebrosides per gram fresh weight. Ninety percent of the glucocere-broside fatty acids were palmitate, stearate and oleate; fatty acids with chain lengths above C20 were virtually absent. No hydroxy fatty acids were found. The long chain bases of adult rat brain glucocerebrosides consisted of 74.6% C18-sphingosine, 24.4% C18-sphinganine and 1.1% C20-sphingosine. These results are compared to those obtained from glucocerebrosides from immature rat brains (Abe & Norton , 1974) and discussed in respect to changes occurring during brain development.  相似文献   

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