Skip to main content
Log in

Assay of plasmalogens and polyunsaturated fatty acids (PUFA) in erythrocytes and fibroblasts

  • Published:
Journal of Inherited Metabolic Disease

Summary

The direct transesterification method of Lepage and Roy is described as used in our laboratory for the analysis of plasmalogens and polyunsaturated fatty acids in erythrocytes and cultured fibroblasts by gas chromatography. An overview is given of the plasmalogen ratios and docosahexaenoic acid concentrations from controls and patients with different peroxisomal disorders investigated in our laboratory.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References

  • Bjorkheim I, Sisfontes L, Boström B, Kase BF, Blomstrand R (1986) Simple diagnosis of the Zellweger syndrome by gas-liquid chromatography.J Lipid Res 27: 786–791.

    Google Scholar 

  • Lepage G, Roy CC (1986) Direct transesterification of all classes of lipids in a one-step reaction.J Lipid Res 27: 114–120.

    Google Scholar 

  • Martinez M (1989) Polyunsaturated fatty acid changes suggesting a new enzymatic defect in Zellweger syndrome.Lipids 24: 261–265.

    Google Scholar 

  • Martinez M (1990) Severe deficiency of docosahexaenoic acid in peroxisomal diseases: a defect of Δ-4 desaturation?Neurology 40: 1292–1298.

    Google Scholar 

  • Martinez M (1992a) Abnormal profiles of polyunsaturated fatty acids in the brain, liver, kidney and retina of patients with peroxisomal disorders.Brain Res 583: 171–182.

    Google Scholar 

  • Martinez M (1992b) Treatment with docosahexaenoic acid favorably modifies the fatty acid composition of erythrocytes in peroxisomal patients. In Coates PM, Tanaka K, eds.New Developments in Fatty Acid Oxidation. New York: Wiley-Liss, 389–397.

    Google Scholar 

  • Martinez M (1994a) Polyunsaturated fatty acids in the developing human brain, red cells and plasma: influence of nutrition and peroxisomal disease. In Galli C, Simopoulos AC, Tremoli E, eds.Fatty Acids and Lipids: Biological Aspects. (World Rev Nutr Diet. 75) Karger: Basel, 70–78.

    Google Scholar 

  • Martinez M (1995) Polyunsaturated fatty acids in the developing human brain, erythrocytes and plasma in peroxisomal disease: therapeutic implications.J Inher Metab Dis 18 (Suppl. 1: 61–75.

    Google Scholar 

  • Martinez M, Mougan I, Ballabriga A (1994b) Blood polyunsaturated fatty acids in patients with peroxisomal disorders: a multicenter study.Lipids 29: 273–280.

    Google Scholar 

  • Wanders RJA, Purvis YR, Heymans HSA, et al. (1986) Age-related differences in plasmalogen content of erythrocytes from patients with the cerebro-hepato-renal (Zellweger) syndrome: implications for postnatal detection of the disease.J Inher Metab Dis 9: 335–342.

    Google Scholar 

  • Wanders RJA, Schumacher H, Heikoop J, Schutgens RBH, Tager JM (1992) Human dihydroxyacetonephosphate acyltransferase deficiency: A new peroxisomal disorder.J Inher Metab Dis 15: 389–399.

    Google Scholar 

  • Wanders RJA, Schutgens RBH, Barth PG, Tager JM, Bosch H vd (1993) Postnatal diagnosis of peroxisomal disorders: a biochemical approach.Biochemie 75: 269–279.

    Google Scholar 

  • Wanders RJA, Dekker C, Horvath VAP et al (1994) Human alkyldihydroxyacetonephosphate synthase deficiency: a new peroxisomal disorder.J Inher Metab Dis 17: 315–318.

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Dacremont, G., Vincent, G. Assay of plasmalogens and polyunsaturated fatty acids (PUFA) in erythrocytes and fibroblasts. J Inherit Metab Dis 18 (Suppl 1), 84–89 (1995). https://doi.org/10.1007/BF00711431

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF00711431

Keywords

Navigation