Linoleic acid supplemention of Barth syndrome fibroblasts restores cardiolipin levels: implications for treatment
| Autor: | Peter G. Barth, A. H. van Gennip, Frédéric M. Vaz, H. Overmars, Fredoen Valianpour, Ron J. A. Wanders |
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| Přispěvatelé: | Paediatric Metabolic Diseases, Laboratory Genetic Metabolic Diseases, Paediatric Neurology |
| Rok vydání: | 2003 |
| Předmět: |
linoleic acid
Spectrometry Mass Electrospray Ionization medicine.medical_specialty Adolescent Cardiolipins Linoleic acid Tafazzin QD415-436 Tandem mass spectrometry Biochemistry chemistry.chemical_compound Endocrinology Mitochondrial myopathy HPLC-ESI-MS/MS Internal medicine medicine Cardiolipin Humans Child Incubation Cells Cultured Chromatography High Pressure Liquid biology Chemistry mitochondrial myopathy Infant Genetic Diseases X-Linked Phosphatidylglycerols Barth syndrome Syndrome Cell Biology Fibroblasts medicine.disease biology.protein lipids (amino acids peptides and proteins) cardiolipin cardiomyopathy After treatment |
| Zdroj: | Journal of Lipid Research, Vol 44, Iss 3, Pp 560-566 (2003) Journal of lipid research, 44(3), 560-566. American Society for Biochemistry and Molecular Biology Inc. |
| ISSN: | 0022-2275 |
| DOI: | 10.1194/jlr.m200217-jlr200 |
| Popis: | The object of this study was to investigate whether the levels of cardiolipin in cultured skin fibroblasts of patients with Barth syndrome (BTHS) can be restored by addition of linoleic acid to growth media. To this end, fibroblasts from controls and BTHS 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 BTHS 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 BTHS patients after treatment with linoleic acid indicate that an increased amount of linoleic acid in the diet might be beneficial to BTHS patients. |
| Databáze: | OpenAIRE |
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