J. Lipid Res. Neurobiology of Lipids (ISSN1683-5506)
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A more recent version of this article appeared on March 1, 2003

Papers In Press, published online ahead of print December 16, 2002
J. Lipid Res., doi:10.1194/jlr.M200217-JLR200
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Submitted on June 4, 2002
Revised on December 12, 2002
Accepted on December 13, 2002

The effect of linoleic acid supplementation on cardiolipin levels in fibroblasts of patients suffering from Barth syndrome; implications for treatment

Fredoen Valianpour, Ronald J. W. Wanders, Henk Overmars, Frédéric M. Vaz, Peter G. Barth, and Albert H. van Gennip

Lab. Genetic Metabolic Diseases, Academic Medical Center, Amsterdam 1105 AZ

Corresponding Author: r.j.wanders{at}amc.uva.nl

The object of this study was to investigate whether the levels of cardiolipin in cultured skin fibroblasts of patients with Barth Syndrome can be restored by addition of linoleic acid to growth media. To this end, fibroblasts from controls and Barth Syndrome patients were grown in the presence or absence of linoleic acid. High Performance Liquid Chromatography-Electrospray Ionization tandem Mass Spectrometry was used for quantitative and compositional analysis of cardiolipin. Incubation of cells from both Barth Syndrome and controls with different concentrations of linoleic acid led to a dose and time-dependent increase of cardiolipin levels. The increased levels of cardiolipin in fibroblasts of Barth Syndrome patients after treatment with linoleic acid indicate that an increased amount of linoleic acid in the diet might be beneficial to BTHS patients.


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