Plasma and red blood cell fatty acids in peroxisomal disorders

Neurochemical Research

Volumn 24, Issue 2, 1999, Pages 187-197

  Moser, Ann B ^b   Jones, David S ^b   Raymond, Gerald V ^b   Moser, Hugo W ^a  

^a KENNEDY KRIEGER INSTITUTE   (United States)

^b NONE

Author keywords

Fatty acids; Infantile Refsum disease; Neonatal adrenoleukodystrophy; Plasmalogens; X linked adrenoleukodystrophy; Zellweger syndrome

Indexed keywords

ARACHIDONIC ACID; DOCOSAHEXAENOIC ACID; FATTY ACID; FISH OIL; LORENZO OIL; OMEGA 3 FATTY ACID; OMEGA 6 FATTY ACID; PLASMALOGEN; SATURATED FATTY ACID; VERY LONG CHAIN FATTY ACID;

ADOLESCENT; ADRENOLEUKODYSTROPHY; ADULT; ARTICLE; BLOOD LEVEL; CHILD; CONTROLLED STUDY; DIET THERAPY; DISORDERS OF PEROXISOMAL FUNCTIONS; ERYTHROCYTE LEVEL; HUMAN; HUMAN CELL; HUMAN TISSUE; INFANT; MAJOR CLINICAL STUDY; MALE; PRIORITY JOURNAL; REFSUM DISEASE; ZELLWEGER SYNDROME;

ADOLESCENT; ADULT; CHILD; CHILD, PRESCHOOL; CHROMATOGRAPHY, GAS; DIETARY FATS, UNSATURATED; DRUG COMBINATIONS; ERUCIC ACIDS; ERYTHROCYTES; FATTY ACIDS; HUMANS; INFANT; MALE; MIDDLE AGED; PEROXISOMAL DISORDERS; TRIOLEIN;

EID: 0032935287     PISSN: 03643190     EISSN: None     Source Type: Journal    
DOI: 10.1023/A:1022549618333     Document Type: Article

References (31)

1. Igarashi, M., Schaumburg, H. H., Powers, J., Kishimoto, Y., Kolodny, E., and Suzuki, K. 1976: Fatty acid abnormality in adrenoleukodystrophy. J. Neurochem. 26:851-860.
2. Mosser, J., Lutz, Y., Stoeckel, M. E., Sarde, C.-O., Kretz, C., Douar, A. M., Lopez, J., Aubourg, P., and Mandel, J. L. 1994. The gene responsible for adrenoleukodystrophy encodes a peroxisomal membrane protein. Hum. Mol. Genet. 3(2):265-271.
3. Moser, H. W. 1997. Adrenoleukodystrophy: Phenotype, genetics, pathogenesis and therapy. Brain. 120:1485-1508.
4. Singh, I., Moser, A. B., Goldfischer, S., and Moser, H. W. 1984. Lignoceric acid is oxidized in the peroxisomes: Implications for the Zellweger cerebro-hepato-renal syndrome and adrenoleukodystrophy. Proc. Natl. Acad. Sci. USA. 81:4203-4207.
5. Moser, A. B., Singh, I., Brown III, F. R., Solish, G. I., Kelly, R. I., Benke, P. J., and Moser, H. W. 1984. The cerebro-hepato-renal (Zellweger) syndrome: Increased levels and impaired degradation of very long chain fatty acids, and prenatal diagnosis. New Engl. J. Med. 310:1141-1145.
6. Wanders, R. J. A., Barth, P. G., Schutgens, R. B. H., and Heymans, H. S. 1996. A. Peroxisomal disorders: Post- and prenatal diagnosis based on a new classification with flowcharts. Int. Pediatr. 11(4):203-214.
7. Mosser, J., Douar, A.-M., Sarde, C.-O., Kioschis, P., Feil, R., Moser, H., Poustka, A.-M., Mandel, J.-M., and Aubourg, P. 1993. Putative X-linked adrenoleukodystrophy gene shares unexpected homology with ABC transporters. Nature. 361:726-730.
8. Martinez, M. 1989. Polyunsaturated fatty acid changes suggesting a new enzymatic defect in Zellweger syndrome. Lipids. 24:261-265.
9. Bazan, N. G. 1990. Supply of n-3 polyunsaturated fatty acids and their significance in the central nervous system. In: Wurtman, R. J. and Wurtman, J. J. (Ed). Nutrition and the Brain. New York: Raven Press; Volume 8 pp. 1-22.
10. Zellweger, H. 1987. The cerebro-hepato-renal (Zellweger) syndrome and other peroxisomal disorders. Dev. Med. Child. Neurol. 29:821-829.
11. Braverman, N., Dodt, G., Gould, S. J., and Valle, D. 1995. Disorders of peroxisome biogenesis. Hum. Mol. Genet. 4:1791-1798.
12. Moore, S. A., Hurt, E., Yoder, E., Sprecher, H., and Spector, A. A. 1995. Docosahexaenoic acid synthesis in human skin fibroblasts involves peroxisomal retroconversion of tetracosahexaenoic acid. J. Lipid. Res. 36:2433-2443.
13. Ho, J. K., Moser, H., Kishimoto, Y., and Hamilton, J. A. 1995. Interaction of very long chain fatty acid with model membranes and serum albumin: Implications for the pathogenesis of adrenoleukodystrophy. J. Clin. Invest. 96:1455-1463.
14. Whitcomb, R. W., Linehan, W. R., and Knazek, R. A. 1988. Effects of long-chain, saturated fatty acids on membrane microviscosity and adrenocorticotropin responsiveness of human adrenocortical cells in vitro. J. Clin. Invest. 81:185-188.
15. Martinez, M. 1993. Abnormal profiles of polyunsaturated fatty acids in the brain, liver, kidney, and retina of patients with peroxisomal disorders. Brain Res. 583:171-182.
16. Martinez, M., Pineda, M., Vidal, R., and Martin, B. 1993. Docosahexaenoic acid: A new therapeutic approach to peroxisomal patients. Experience with two cases. Neurology. 43:1389-1397.
17. Rizzo, W. B., Leshner, R. T., Odone, A., Dammann, A. L., Craft, D. A., Jensone, M. E., Jennings, S. S., Davis, S., Jaitly, R., and Sgro, J. A. 1989. Dietary erucic acid therapy for X-linked adrenoleukodystrophy. Neurology. 39(11): 1415-1422.
18. Odone, A. and Odone M. Lorenzo's oil. 1989. A new treatment for adrenoleukodystrophy. J. Pediatr. Neurosci. 5:55-61.
19. Martinez, M. 1990. Severe deficiency of docosahexaenoic acid in peroxisomal disorders: A defect of delta-4 desaturations? Neurology. 40:1292-1298.
20. Lepage, G. and Roy, C. C. 1986. Direct transesterification of all classes of lipids in a one-step reaction. J. Lipid Res. 27:114-120.
21. Martinez, M., Mougan, I., Roig, M., and Ballabriga, A. 1994. Blood polyunsaturated fatty acids in patients with peroxisomal disorders. A multicenter study. Lipids. 29:273-280.
22. Moser, A. B., Rasmussen, M., Naidu, S., Watkins, P. A., McGuinness, M., Hajra, A. K., Chen, G., Raymond, G., Liu, A., Gordon, D., Garnaas, K., Walton, D. S., Skjeldal, O. H., Guggenheim, M. A., Jackson, L. G., Elias, E. R., and Moser, H. W. 1995. Phenotype of patients with peroxisomal disorders subdivided into 16 complementation groups. J. Pediatr. 127:13-22.
23. Moser, H. W. and Borel, J. 1995. Dietary therapy for adrenoleukodystrophy. Annu. Rev. Nutr. 15:379-397.
24. Lepage, G., Levy, E., Ronco, N., Smith, L., Galeano, N., and Roy, C. C. 1989. Direct transesterification of plasma fatty acids for the diagnosis of essential fatty acid deficiency in cystic fibrosis. J. Lipid. Res. 30:1484-1490.
25. Moser, H. W., Smith, K. D., and Moser, A. B. 1994. X-linked Adrenoleukodystrophy. Pages 2325-2349; in Scriver, C. R., Beaudet, A. L., Sly, W. S., and Valle, D. (eds). The Metabolic and Molecular Basis of Inherited Disease. Seventh Edition. New York: McGraw Hill; (Chapter 72).
26. Holman, R. T. 1960. The ratio of trienoic-tetranoic acids in tissue lipids as a measure of essential fatty acid requirement. J. Nutr. 70: 405.
27. Decsi, T. and Koletsko, B. 1994. Fatty acid composition of plasma lipid classes in healthy subjects from birth to young adulthood. Eur. J. Pediatr. 153:520-525.
28. Innis, S. M. 1991. Essential fatty acids in growth and development. Prog. Lipid. Res. 30(1):39-103.
29. Moser, H. W. and Moser, A. B. 1996. Very long chain fatty acids in diagnosis, pathogenesis, and therapy of peroxisomal disorders. Lipids. 31(Suppl):S141-S145.
30. Avoldano, M. I. and Donnari, D. 1996. Plasma phospholipid fatty acids in X-linked adrenoleukodystrophy. Clin. Chem. 42:454-461.
31. Martinez, M. 1992. Treatment with docosahexaenoic acid favorably modifies the fatty acid composition of erythrocytes in peroxisomal patients. In: Coates, P. M. and Tanaka, K. (Eds). Proceedings of the Second International Symposium on Clinical, Biochemical, and Molecular Aspects of Fatty Acid Oxidation. New York: Wiley-Liss; pp. 389-397.