Valianpour F, Wanders R J A, Overmars H, Vaz F M, Barth P G, van Gennip A H
Academic Medical Center, University of Amsterdam, Laboratory Genetic Metabolic Diseases, Emma Children's Hospital and Department of Clinical Chemistry, PO Box 22700, 1100 DE Amsterdam, The Netherlands.
J Lipid Res. 2003 Mar;44(3):560-6. doi: 10.1194/jlr.M200217-JLR200. Epub 2002 Dec 16.
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.
本研究的目的是调查在巴思综合征（BTHS）患者的培养皮肤成纤维细胞中，通过在生长培养基中添加亚油酸是否可以恢复心磷脂水平。为此，将来自对照组和BTHS患者的成纤维细胞在有或没有亚油酸的情况下培养。采用高效液相色谱 - 电喷雾电离串联质谱法对心磷脂进行定量和成分分析。用不同浓度的亚油酸孵育BTHS患者和对照组的细胞，导致心磷脂水平呈剂量和时间依赖性增加。用亚油酸处理后，BTHS患者成纤维细胞中心磷脂水平的增加表明饮食中增加亚油酸的量可能对BTHS患者有益。
