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Plasmalogens and Polyunsaturated Fatty Acids

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Laboratory Guide to the Methods in Biochemical Genetics

Abstract

The analysis of polyunsaturated fatty acids (“essential fatty acids”) in plasma and erythrocytes is carried out by gas chromatography of their methyl esters following trans-methylation of the fatty acid esters. The O-alkenyl ethers or plasmalogens – an important group of phospholipids – are converted into dimethylacetals in this process and can be analyzed in the same analysis. Decreased levels of essential fatty acids are associated with various psychiatric disorders as well as defects of peroxisomal fatty acid beta-oxidation. The nutritional status of the patient may play an additional role. Defective plasmalogen biosynthesis may be the consequence of isolated peroxisomal enzyme deficiencies or peroxisome biogenesis defects. Plasmalogens are the key metabolites in the diagnosis of rhizomelic chondrodysplasia punctata.

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Authors and Affiliations

  1. Laboratory Genetic Metabolic Diseases, Academic Medical Center, University of Amsterdam, 22700, 1100 DE, Amsterdam, Netherlands

    Marinus Duran

  2. Department of Pediatrics and Clinical Chemistry, AMC, University Hospital Amsterdam, Meibergdreef 9, 1105 AZ, Amsterdam, Netherlands

    Ronald J.A. Wanders

Authors
  1. Marinus Duran
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  2. Ronald J.A. Wanders
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Editor information

Editors and Affiliations

  1. Division of Clinical Chemistry and Biochemistry, University Children’s Hospital, Steinwiesstrasse 75, 8032, Zurich, Switzerland

    Nenad Blau

  2. Academic Medical Centre, Lab. Genetic Metabolic; Diseases Fo-224, University Amsterdam, Meibergdreef 9, 1105 AZ, Amsterdam, Netherlands

    Marinius Duran

  3. Dept. of Molecular and Medical Genetics, Oregon Health & Science University, 3rd Avenue 2525, 97201, Portland, USA

    K. Michael Gibson

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© 2008 Springer-Verlag Berlin Heidelberg

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Duran, M., Wanders, R. (2008). Plasmalogens and Polyunsaturated Fatty Acids. In: Blau, N., Duran, M., Gibson, K. (eds) Laboratory Guide to the Methods in Biochemical Genetics. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-76698-8_11

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  • DOI: https://doi.org/10.1007/978-3-540-76698-8_11

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-540-76697-1

  • Online ISBN: 978-3-540-76698-8

  • eBook Packages: MedicineMedicine (R0)

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