Hostname: page-component-745bb68f8f-v2bm5 Total loading time: 0 Render date: 2025-01-26T06:30:48.593Z Has data issue: false hasContentIssue false
  • English
  • Français

Fatty Acid Profile of Major Lipid Classes in Plasma Lipoproteins of Patients with Friedreich's Ataxia — Demonstration of a Low Linoleic Acid Content most evident in the Cholesterol-Ester Fraction

Published online by Cambridge University Press:  18 September 2015

Jean Davignon ,
Y.S. Huang ,
J.P. Wolf  and
A. Barbeau
Jean Davignon
Affiliation:
Department of Lipid Metabolism and Atherosclerosis Research, and the Department of Neurobiology, Clinical Research Institute of Montreal
Y.S. Huang
Affiliation:
Department of Lipid Metabolism and Atherosclerosis Research, and the Department of Neurobiology, Clinical Research Institute of Montreal
J.P. Wolf
Affiliation:
Department of Lipid Metabolism and Atherosclerosis Research, and the Department of Neurobiology, Clinical Research Institute of Montreal
A. Barbeau
Affiliation:
Department of Lipid Metabolism and Atherosclerosis Research, and the Department of Neurobiology, Clinical Research Institute of Montreal
Rights & Permissions [Opens in a new window]

Summary

Core share and HTML view are not available for this content. However, as you have access to this content, a full PDF is available via the ‘Save PDF’ action button.

Studies were undertaken to further characterize plasma lipids and lipoprotein abnormalities in Friedreich's ataxia. The high density lipoprotein (HDL) apo AI/All ratio was quantitated by densitometry and found to be normal. The free to esterified cholesterol ratio in HDL was lower in Friedreich's ataxia because of a reduction in the amount of free cholesterol in this lipoprotein class. The fatty acid profile of the cholesteryl-ester (CE) fraction was markedly deficient in linoleic acid (18:2) in both total plasma and HDL· There was a compensatory increase in saturated acids. The HDL phospholipid (PL) fraction also showed a reduction in the proportion of 18:2 with a concomitant increase in stearic (18:0) and oleic acid (18:1) while the HDL triglycéride (TG) fraction showed only an increase in palmi t oleic (16:1) and oleic acids. Feeding of soya lecithin rich in 18:2 failed to increase significantly the 18:2 content of HDL-CE and HDL-PL but lowered the percentage of 16:1 and 18:1 in all 3 lipid classes of HDL. Although the total plasma CE fatty acid profile was perturbed in Friedreich's Ataxia, total plasma PL and TG fatty acid patterns were unaffected. Among the plasma lipoprotein fatty acid profiles, that of the low density lipoprotein (LDL) was most affected, then that of the HDL. The very low density lipoprotein (VLDL) fatty acid composition showed an increase in 16:1 and a decrease in 18:2 which were entirely corrected by lecithin feeding. These results suggest the existence of a metabolic defect in the incorporation of 18:2 into chylomicron phospholipids within the intestinal mucosa.

Type
Research Article
Information
Canadian Journal of Neurological Sciences , Volume 6 , Issue 2 , May 1979 , pp. 275 - 283
Copyright
Copyright © Canadian Neurological Sciences Federation 1979

References

Baker, R. W. R.Thompson, R. H. S. and Zilkha, K. J. (1963). Fatty acid composition of brain lecithins in multiple sclerosis. Lancet 1, 26–27.CrossRefGoogle ScholarPubMed
Barbeau, A. (1976). Friedreich'sataxia 1976 An overview. Can. J. Neurol. Sci. 3, 389–397.Google ScholarPubMed
Bartlett, G. R.(1959). Phosphorus assay in column chromatography. J. Biol. Chem. 234, 466–468.CrossRefGoogle ScholarPubMed
Block, W. D., Jarrett, K. J., and Levine, J. B. (1966). An improved automated determination of serum total cholesterol with a single color reagent. Clin. Chim. 12, 681–689.CrossRefGoogle ScholarPubMed
Cheung, M. C. and Albers, J. J. (1977). The measurement of apolipoprotein AI and All levels in men and women by immunoassay. J. Clin, invest 60, 43–50.Google ScholarPubMed
Folch, J., Lees, M., and Sloane-Stanley, G. H. (1957). A simple method for the isolation and purification of total lipids from animal tissues. J. Biol. Chem. 226, 497–509.CrossRefGoogle ScholarPubMed
Goodman, D. S. and Shiratori, T. (1964). Fatty acid composition of human plasma lipoprotein fractions. J. Lipid Res. 5, 307–313.CrossRefGoogle ScholarPubMed
Havel, R. J., Eder, H. A. and Bragdon, J. H. (1955). The distribution and chemical composition of ultracentrifugally separated lipoproteins in human serum. J. Clin. Invest. 34, 1345–1353.CrossRefGoogle ScholarPubMed
Herbert, P. N., Gotto, A. G., and Fredrickson, D. S. (1978). Familial lipoprotein deficiency (abetalipoprotein-emia, hypobetalipoproteinemia and Tangier disease).In: The metabolic basis of inherited disease. 4th edition, (Stanbury, J. B., Wyngaarden, J. B. and Fredrickson, D. S. eds). McGraw-Hill, New York, pp. 544–588.Google Scholar
Huang, Y. S., Nestruck, A. C., Barbeau, A., Bouchard, J. P. and Davignon, J. (1978). Plasma lipids and lipoproteins in Friedreich's ataxia and familial spastic ataxia — Evidence for an abnormal composition of high density lipoproteins. Can. J. Neurol. Sci. 5, 149–156.Google ScholarPubMed
Kalofoutis, A. and Jullien, G. (1974). A study of serum fatty acids in neurological diseases. Biochimie 56, 623–624.CrossRefGoogle ScholarPubMed
Kane, J. P. (1973). A rapid electrophoretic technique for identification of subunit species of apoprotein in serum lipoproteins. Anal. Biochem. 53, 350–364.CrossRefGoogle ScholarPubMed
Kane, J. P., Sata, T., Hamilton, R. L. and Havel, R. J. (1975). Apoprotein composition of very low density lipoproteins of human serum. J. Clin. Invest. 56, 1622–1634.CrossRefGoogle ScholarPubMed
Kraml, N. and Cosyns, L. (1969). A semi-automated determination of serum triglycérides. Clin. Biochem. 2, 373–380.CrossRefGoogle Scholar
Laurell, S. (1966). Method for routine determination of plasma triglycérides. Scand. J. Clin. Lab. Invest. 18, 668–672.CrossRefGoogle ScholarPubMed
Marcel, Y. L. and Vez1Na, C. (1973). A method for the determination of the initial rate of reaction of lecithin: cholesterol acyltransferase in human plasma. Biochim. Biophys. Acta 306, 497–504.CrossRefGoogle ScholarPubMed
Morrison, W. R. and Smith, L. M. (1964). Preparation of fatty acid methyl esters and dimethylacetals from lipids in the boron fluoride-methanol. J. Lipid Res. 5, 600–608.CrossRefGoogle ScholarPubMed
Reed, C. F. (1968). Phospholipid exchange between plasma and erythrocytes in man and the dog. J. Clin. Invest. 47, 749–760.CrossRefGoogle ScholarPubMed
Renooij, W. and Van Golde, L. M. G. (1977). The transposition of molecular classes of phosphatidylcholine across the rat erythrocyte membrane and their exchange between the red cell membrane and plasma lipopŕoteins. Biochim. Biophys. Acta 470, 405–474.CrossRefGoogle ScholarPubMed
Rosseneu, M., Declercq, B., Vano-Amme, D., Vercaemst, R., Soete-Wey, F., Peeters, H. and Blaton, V. (1979). Influence of oral polyunsaturated and saturated phospholipid treatment on the lipid composition and fatty acid profile of chimpanzee lipopŕoteins. Atherosclerosis 32, 141–153.CrossRefGoogle ScholarPubMed
Sandbank, U., Bechar, M. and Born-Stein, B. (1971). Hyperlipemic polyneuro-pathy. Acta Neuropath. 19, 290–300.CrossRefGoogle Scholar
Scanu, A. M. (1978). Abetalipoproteinemia and hypobetalipoproteinemia: what is the primary defect? Advances Neurol. 21, 125–130.Google Scholar
Schaefer, E. J., Blum, C. B., Levy, R. L., Jenkins, L. L., Alaupovic, P., Foster, D. M., Brewer, H. B. Jr. (1978). Metabolism of high-density lipopro-tein apolipoproteins in Tangier disease. New Engl. J. Med. 299, 905–910.CrossRefGoogle Scholar
Shepherd, J., Packard, C. J., Patsch, J. R., Gotto, A. M. Jr. and Taunton, O. D. (1978). Effects of dietary polyunsaturated and saturated fat on the properties of high density lipopŕoteins and the metabolism of apolipoprotein AI. J. Clin. Invest. 61, 1582–1592.CrossRefGoogle Scholar
Sidbury, J. B. Jr., Smith, E. K. and Harlan, W. (1967). An inborn error of short-chain fatty acid metabolism. J. Pediatrics 70, 8–15.CrossRefGoogle ScholarPubMed
Spritz, N. and Mishkel, M. A. (1969). Effects of dietary fats on plasma lipids and lipopŕoteins: an hypothesis for the lipid-lowering effect of unsaturated fatty acids. J. Clin. Invest. 48, 78–86.CrossRefGoogle ScholarPubMed
Steinberg, D. (1972). Phytanic acid storage disease: Refsum's syndrome.In: The metabolic basis of inherited disease, 4th edition, (Stanbury, J. B., Wyngaarden, J. B. and Fredrickson, D. S.,eds.). McGraw-Hill, New York, pp. 688–706.Google Scholar
Tall, A. R. and Small, D. M. (1978). Plasma high density lipopŕoteins. New Engl. J. Med. 299, 1232–1236.CrossRefGoogle ScholarPubMed
Yao, J. K.Ellefson, R. D. and Dyck, P. J. (1976). Lipid abnormalities in hereditary neuropathy: Part I. Serum non-polar lipids. J. Neurol. Sci. 29, 161–175.Google ScholarPubMed