Linoleic acid supplemention of Barth syndrome fibroblasts restores cardiolipin levels: implications for treatment
Linoleic acid supplemention of Barth syndrome fibroblasts restores cardiolipin levels: implications for treatment
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DOI：
10.1194/jlr.m200217-jlr200
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发表时间：
2003-03-01
影响因子：
6.5
通讯作者：
van Gennip, AH
中科院分区：
文献类型：
--
作者：
Valianpour, F;Wanders, RJA;van Gennip, AH
本研究的目的是探讨在培养的Barth综合征(BTHS)患者皮肤成纤维细胞中加入亚油酸能否恢复其心磷脂水平。为此，对照组和BTHS患者的成纤维细胞在亚油酸存在或不存在的情况下培养。采用高效液相色谱-电喷雾电离串联质谱法对心磷脂进行了定量和成分分析。BTHS和对照组的细胞与不同浓度的亚油酸孵育后，心磷脂水平呈剂量和时间依赖性增加。BTHS患者经亚油酸治疗后成纤维细胞心磷脂水平升高，提示饮食中增加亚油酸可能对BTHS患者有益。
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.