scholarly journals Erythrocyte Membrane Lipids in Fríedreich's Ataxia

Author(s):  
P. Draper ◽  
Y.S. Huang ◽  
D. Shapcott ◽  
B. Lemieux ◽  
M. Brennan ◽  
...  

SummaryIn a study of the lipid composition of erythrocyte membranes in Friedreich's ataxia, the concentration of the major membrane components (phosr pholipids, cholesterol and protein) in ataxie patients, family members, and control subjects were found to be the same. The total fatty acid distribution was also normal. However, an altered distribution of phospholipid classes in erythrocytes was noted (an increase of PI + PS and a decrease of P E in Friedreich's ataxia patients).

Author(s):  
B. Lemieux ◽  
A. Barbeau ◽  
V. Beroniade ◽  
D. Shapcott ◽  
G. Breton ◽  
...  

SUMMARY:A study of amino acids determined by sequential Multi-sample Amino Acid Automatic Analyzer in plasma, urine and cerebrospinal fluid (CSF) in patients with Friedreich's ataxia and control subjects has revealed a number of mathematically significant variations from normal. Of practical physiological importance are the following: a high urinary excretion of alanine with slightly elevated plasma levels; a low plasma and CSF concentration of aspartic acid in the resence of normal urinary values and finally a low CSF concentration of taurine accompanied by normal plasma levels, but elevated urinary output and renal clearance rates. We postulate that the modifications in alanine and aspartic acid are less specific and probably secondary, but there could be a genetic defect in the membrane transport of taurine and the other β-amino acids in Friedreich's ataxia.


1968 ◽  
Vol 109 (1) ◽  
pp. 51-59 ◽  
Author(s):  
G. G. Forstner ◽  
K. Tanaka ◽  
K. J. Isselbacher

1. Rat intestinal microvillus plasma membranes were prepared from previously isolated brush borders and the lipid composition was analysed. 2. The molar ratio of cholesterol to phospholipid was greatest in the membranes and closely resembled that reported for myelin. 3. Unesterified cholesterol was the major neutral lipid. However, 30% of the neutral lipid fraction was accounted for by glycerides and fatty acid. 4. Five phospholipid components were identified and measured, including phosphatidylethanolamine, phosphatidylcholine, phosphatidylserine, sphingomyelin and lysophosphatidylcholine. Though phosphatidylethanolamine was the chief phospholipid, no plasmalogen was detected. 5. In contrast with other plasma membranes in the rat, the polar lipids of the microvillus membrane were rich in glycolipid. The cholesterol:polar lipid (phospholipid+glycolipid) ratio was about 1:3 for the microvillus membrane. Published data suggest that this ratio resembles that of the liver plasma membrane more closely than myelin or the erythrocyte membrane. 6. The fatty acid composition of membrane lipids was altered markedly by a single feeding of safflower oil. Membrane polar lipids did not contain significantly more saturated fatty acids than cellular polar lipids. Differences in the proportion of some fatty acids in membrane and cellular glycerides were noted. These differences may reflect the presence of specific membrane glycerides.


2018 ◽  
Vol 29 (2) ◽  
pp. 49-55 ◽  
Author(s):  
Albena Merdzhanova ◽  
Veselina Panayotova ◽  
Diana A. Dobreva ◽  
Rostitsa Stancheva ◽  
Katya Peycheva

Abstract Rapana venosa is an edible mollusc with nutritional and economic importance. There is limited information about its lipid composition. The aim of the present study is to provide information about lipid composition, fatty acid profiles, fat soluble vitamins and cholesterol content of raw and cooked Rapana venosa. Cooking did not affect the ratio of lipid classes, but fatty acids composition varied significantly. Considerable variations were observed in fatty acid distribution of total lipids and neutral lipids. Fatty acid groups of phospholipids remained unaffected by temperature treatment. The most abundant fatty acids in all lipid classes of raw and cooked specimens were palmitic acid (C16:0) and eicosapentaenoic acid (C20:5n-3). The sum of omega-3 polyunsaturated fatty acids (PUFA) was higher than omega-6 PUFA in all lipid fractions. The results of the present study showed that cooking process affected cholesterol, fat soluble vitamins and carotenoids content differently. Larger variations were observed for vitamin A, β-carotene and astaxanthin and to lesser for vitamin E. Cholesterol and vitamin D3 were also affected by the thermal stress. The present study revealed that Rapana venosa meat could be a good source of high quality nutritional lipids, which are well preserved even after culinary treatment


Author(s):  
Y.S. Huang ◽  
Y.L. Marcel ◽  
C. Vezina ◽  
A. Barbeau ◽  
J. Davignon

SUMMARY:In a study of the fatty acid composition of erythrocyte membrane phospholipids in Friedreich's ataxia, a lower percentage of linoleic acid in phosphatidylcholine was demonstrated. An enzyme involving the exchange of lipids between plasma and erythrocyte membrane, lecithin: cholesteryl acyltransferase (LCAT) was also studied. It was found that the LCA Tactivity had a trend towards low values. However, crossing-over studies indicated that when the LCAT enzyme of patients was exposed to its own substrate it gave low activity values but that the result reverted to normal when control substrate was used


1980 ◽  
Vol 8 (1) ◽  
pp. 74-76 ◽  
Author(s):  
Janet L. Walker ◽  
Susan Chamberlain ◽  
Norman Robinson

1992 ◽  
Vol 68 (2) ◽  
pp. 435-443 ◽  
Author(s):  
K. M. Abu-Salah ◽  
A. A. Alothman ◽  
K. Y. Lei

The influence of dietary copper on lipid composition, phospholipid-fatty acid and protein profiles and fluidity of the erythrocyte membranes of rats is reported. In general Cu deficiency in rats induced some changes in the phospholipid-fatty acid profile of erythrocyte membranes when compared with Cu-adequate animals. Stearic (18:0) and docosadienoic (22:2n-3) acids contents, for example, were significantly increased (P< 0.001) while oleic (18:1n-9) and linolenic (18:3n-3) acid contents were significantly depressed (P< 0.001) as a result of Cu deficiency. Moreover the cholesterol:phospholipids molar ratio and the cholesterol (mol):membrane proteins (mg) ratio in Cu-deficient rats were, to different degrees, significantly lower than in animals fed on Cu-adequate diets. In addition, diets deficient in Cu led to a reduction in erythrocyte membrane fluidity (P< 0.001) as assessed by the intramolecular excimer fluorescence of 1,3-di(1-pyrenyI) propane. However, no significant alteration in the phospholipid:protein ratio was observed as a result of differences in dietary treatment. The pattern of erythrocyte membrane proteins obtained with sodium dodecyl sulphate-polyacrylamide gel electro-phoresis did not seem to be influenced by Cu-deficient diets.


1978 ◽  
Vol 56 (11) ◽  
pp. 1036-1041 ◽  
Author(s):  
G. H. Rank ◽  
A. J. Robertson ◽  
H. Bussey

Four different plasma membrane preparations were isolated from multiple drug resistant and sensitive isolates of two isogenic groups of Saccharomyces cerevisiae strains: zymolyase ghosts, concanavalin A ghosts, pH 4 nonaggregated vesicles, and sucrose-gradient purified vesicles. The viscosities of these preparations were determined by the use of a fluorescence polarization technique with 1,6-diphenyl-1,3,5-hexatriene.The viscosities of all four membrane preparations within an isogenic set were the same for resistant and sensitive strains. A comparison of the viscosity of zymolyase ghost liposomes showed that zymolyase ghost (glyco) proteins of resistant and sensitive strains had the same effect on viscosity.There was no difference between resistant and sensitive isolates in the mole concentration of the following lipid classes extracted from zymolyase ghosts: phospholipid, sterol, sterol ester, triglyceride, diglyceride, and free fatty acid. The fatty acid distribution of esterified and free fatty acids and the distribution of nine phospholipids was the same in zymolyase ghosts from sensitive and resistant strains.It was concluded that multiple drug resistance does not result from an alteration in plasma membrane viscosity or lipid composition.


Hydrolysis of phospholipid in erythrocyte membranes by phospholipase C ( Cl. perfringens or B. cereus ) leads to the formation of membrane-attached droplets which contain at least some of the lipid products of hydrolysis and possibly other membrane lipids which can no longer be retained in the phospholipiddepleted membranes. A similar effect is observed when isolated membrane lipids are treated with phospholipase C and appears to represent a phase separation resulting from the change in lipid composition. Droplets and residual membranes have been isolated from phospholipase C-modified preparations for chemical analysis. The structural parameters of the residual membranes as observed by electron microscopy and by low angle X-ray diffraction appear to be little changed. This suggests that the residual lipid is able to condense to maintain molecular continuity within the membrane and that the protein components are also able to condense laterally so as not to change the membrane thickness, although they probably do contribute to a change in electron-density distribution which is detected in the X-ray diffraction studies. These observations are further discussed in relation to recent reports on molecular parameters of specific membrane components and suggestions concerning molecular distributions within the membrane.


Author(s):  
P. Wong ◽  
A. Barbeau

SUMMARY:Proteins of human erythrocyte membranes of Friedrich's ataxia patients and controls were examined by SDS-polyacrylamide gel electrophoresis before and after reduction withβ-mercaptoethanol. No difference could be detected in the composition of their state of aggregation. The protein kinase activity of human erythrocyte membranes of eleven Friedreich's ataxia patients and six controls was determined. No difference in their protein kinase activity could be detected. These results are discussed with respect to an involvement of a generalized membrane defect in Friedreich's ataxia.


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