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Serum fatty acids and peroxidase abnormalities in batten's disease

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Research in Experimental Medicine

Summary

The linoleic acid content of serum lipids was measured in 10 patients with Batten's disease and 11 healthy control subjects by gas liquid chromatography. The fatty acid patterns of serum lipids in this disease may reflect a primary or secondary deficiency in essential fatty acid (linoleic acid) probably related to a glutathione peroxidase abnormality.

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References

  1. Alfin-Slater, R. B., Aftergood, L.: Essential fatty acids reinvestigated. Physiol. Rev.48, 758–784 (1968)

    PubMed  Google Scholar 

  2. Anderson, R. E., Maude, M. B.: Phospholipids of bovine rod outer segments. Biochemistry9, 3624–3628 (1970)

    PubMed  Google Scholar 

  3. Anderson, R. E., Sperling, L.: Lipids of ocular tissues. VII. Positional distribution of fatty acids in the phospholipids of bovine retina rod outer segments. Arch. Biochem. Biophys.144, 673–677 (1971)

    PubMed  Google Scholar 

  4. Anderson, R. E., Maude, M. B.: Lipids of ocular tissues. VIII. The effects of essential fatty acid deficiency on the phospholipids of the photoreceptor membranes of the rat retina. Arch. Biochem. Biophys.151, 270–276 (1972)

    PubMed  Google Scholar 

  5. Anderson, R. E., Landis, D. J., Dudley, P. A.: Essential fatty acid deficiency and renewal of rod outer segments in the albino rat. Invest. Ophthalmol.15, 232–239 (1976)

    PubMed  Google Scholar 

  6. Armstrong, D., Dimmitt, S., Brehme, D. H., Leonberg, S. C., Vogel, W.: Leukocyte peroxidase deficiency in a family with a dominant form of Kuf's disease. Science186, 155–156 (1974)

    PubMed  Google Scholar 

  7. Armstrong, D. S., Dimmitt, S., VanWormer, D. E.: Studies in Batten disease. Arch. Neurol.30, 144–152 (1974)

    PubMed  Google Scholar 

  8. Batten, F. E.: Cerebral degeneration with symmetrical changes in the maculae in two members of a family. Trans. Ophthalmol. Soc. U.K.23, 386–390 (1903)

    Google Scholar 

  9. Bergström, S., Samuelsson, B.: The prostaglandins. Endeavour27, 109–113 (1968)

    PubMed  Google Scholar 

  10. Burr, G. O., Burr, M. M.: New deficiency disease produced by rigid exclusion of fat from the diet. J. Biol. Chem.82, 345–367 (1929)

    Google Scholar 

  11. Collins, F. D., Sinclair, A. J., Royle, J. P., Coats, D. A., Maynard, A. T., Leonard, R. F.: Plasma lipids in human linoleic acid deficiency. Nutr. Metab.13, 150–167 (1971)

    PubMed  Google Scholar 

  12. Combes, M. A., Pratt, E. L., Wiese, M. F.: Essential fatty acids in primature infant feeding. Paediatrics30, 136 (1962)

    Google Scholar 

  13. Egeskov Jensen, G., Clausen, J., Melchior, J. C., Konat, G.: Clinical, Social and Biochemical studies on Batten's syndrome, alias Spielmeyer-Vogter-Stengel's syndrome. Eur. Neurol.15, 203–211 (1977)

    PubMed  Google Scholar 

  14. Egeskov Jensen, G., Shukla, V. K. S., Nielsen, G. G., Clausen, J.: Biochemical abnormalities in Batten's disease. Scand. J. Clin. Lab. Invest. (in press, 1978)

  15. Flohe, L., Gunzler, W. A., Ladenstein, R.: Glutathione peroxidase. In: Glutathione metabolism and function, I. M. Arias, W. B. Jacoby, eds., pp. 115–135. New York: Raven Press 1976

    Google Scholar 

  16. Geigy: Documenta Geigy, Scientific tables, Geigy, Basel, Switzerland 1965

  17. Hamberg, M., Samuelsson, B.: Prostaglandins endoperoxides. Novel transformations of arachidonic acid in human platelets. Proc. Natl. Acad. Sci. USA71, 3400–3404 (1976)

    Google Scholar 

  18. Houtsmuller, U. M. T.: Specific biological effects of polyunsaturated fatty acids. In: The role of fats in human nutrition, A. J. Vergoesen, ed., pp. 331–351. London: Academic Press 1975

    Google Scholar 

  19. Kingsburg, R. J., Brett, C., Stovald, R., Chapman, A., Anderson, J., Morgan, D. M.: Abnormal fatty acid composition and human atherosclerosis. Postgrad. Med. J.50, 425–440 (1974)

    PubMed  Google Scholar 

  20. Naismith, D. J.: Kwashiorkor in Western Nigeria: a study of traditional weaning foods with particular reference to energy and linoleic acid. Br. J. Nutr.30, 567–576 (1973)

    PubMed  Google Scholar 

  21. Nelson, G. J.: Isolation and purification of lipids from animal tissues. In: Analysis of lipids and lipoproteins, E. G. Perkin, ed., pp. 1–22. Champaign, Ill.: Am. oil. Chem. Soc. 1975

    Google Scholar 

  22. Nugteren, D. M.: Arachidonate lipoxygenase in blood platelets. Biochim. Biophys. Acta380, 299–307 (1975)

    PubMed  Google Scholar 

  23. Paglia, D. E., Valentine, W. N.: Studies on the qualitative characterization of erythrocyte glutathione peroxidase. J. Lab. Clin. Med.70, 158–169 (1970)

    Google Scholar 

  24. Paulsrud, J. R., Pensler, L., Whitten, C. F., Stewart, S., Holman, R. T.: Essential fatty acid deficiency in infants induced by fatfree intravenous feeding. Am. J. Clin. Nutr.25, 897–904 (1972)

    PubMed  Google Scholar 

  25. Press, M., Hartop, P. J., Prottey, C.: Correction of essential fatty acid deficiency by the cutaneous application of sunflowerseed oil. Lancet1974 I, 597–599

  26. Shukla, V. K. S., Clausen, J., Larsen, E., Egsgaard, H.: The content of fat and polyenoic acids in the major food sources of arctic diet: Localization of double bonds in fatty acids by means of mass spectrometry of fatty acid pyrrolipides. Lipids (in press, 1977)

  27. Shimonyama, T., Kikuchi, M., Press, M., Thompson, G. R.: Fatty acid composition of plasma lipoproteins in control subjects and in patients with malabsorption. Gut14, 716–722 (1973)

    PubMed  Google Scholar 

  28. Spielmeyer, N.: Weitere Mitteilung über eine besondere Form von familiärer amaurotischer Idiotie. Neurol. Zbl.24, 1131–1132 (1905)

    Google Scholar 

  29. Stengel, C.: En beretning om et mærkeligt sygdomstilfælde hos fire syskende i nærheden af Røraas Eid (Kristiania) 347 (1826)

  30. Van Dorp, D. A.: Essential fatty acids and prostaglandins. Acta Biol. Med. Ger.35, 1041–1049 (1976)

    PubMed  Google Scholar 

  31. Vergroesen, A. J.: Early signs of polyunsaturated fatty acid deficiency. Bibl. Nutr. Dieta23, 19–26 (1976)

    PubMed  Google Scholar 

  32. Vogt, H.: Über familiäre amaurotische Idiotie und verwandte Krankheitsbilder. Mschr. Psychiat. Neurol.18, 310–357 (1905)

    Google Scholar 

  33. Whaun, J. M., Oski, F. A.: Relations of red blood cell glutathione peroxidase to neonatal jaundice. J. Pediat.76, 555–560 (1970)

    PubMed  Google Scholar 

  34. Wiese, H. F.: In: The Vitamins, W. H. Sebrell, R. S. Harris, eds., pp. 327–335. New York: Academic Press 1971

    Google Scholar 

  35. Wolfe, L. S., Kin, N. M. K. N. Y., Baker, R. R., Carpenter, S., Andermann, F.: Identification of retinoyl complexes as the autofluorescent component of the neuronal storage material in Batten disease. Science195, 1360–1362 (1977)

    PubMed  Google Scholar 

  36. Zeman, W.: Studies in the neuronal ceroid lipofuscinosis. J. Neuropath. Expt. Neurol.33, 1–12 (1974)

    Google Scholar 

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Authors and Affiliations

  1. Laboratory of Biochemistry and Toxicology, Institute of Life Sciences, Roskilde Universitetscenter, P. Box 260, DK-4000, Roskilde, Denmark

    V. K. S. Shukla, G. Egeskov Jensen & J. Clausen

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  1. V. K. S. Shukla
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  2. G. Egeskov Jensen
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  3. J. Clausen
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Shukla, V.K.S., Jensen, G.E. & Clausen, J. Serum fatty acids and peroxidase abnormalities in batten's disease. Res. Exp. Med. 173, 27–34 (1978). https://doi.org/10.1007/BF01851371

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  • DOI: https://doi.org/10.1007/BF01851371

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