Aubourg P, Bougnères P F, Rocchiccioli F
J Lipid Res. 1985 Feb;26(2):263-7.
In order to quantify accurately the plasma content of very long chain fatty acids, we have developed a selected ion monitoring gas-liquid chromatographic-mass spectrometric micromethod which allows all of these acids (22:0, 24:1, 24:0, 26:1, and 26:0) to be determined simultaneously in the same 0.5-ml plasma sample; 17:0 and 27:0 fatty acids are used as assay internal standards. For plasma samples in the range equivalent to the various very long chain fatty acid physiological concentrations, assay precision was +/- 2%. The present method has been successfully applied to the biological recognition of patients with adrenoleukodystrophy, their heterozygote relatives, and of cerebro-hepato-renal syndrome and neonatal adrenoleukodystrophy.
为了准确量化极长链脂肪酸的血浆含量，我们开发了一种选择离子监测气-液色谱-质谱微量法，该方法可在同一份0.5毫升血浆样本中同时测定所有这些脂肪酸（22:0、24:1、24:0、26:1和26:0）；17:0和27:0脂肪酸用作测定内标。对于相当于各种极长链脂肪酸生理浓度范围的血浆样本，测定精密度为±2%。本方法已成功应用于肾上腺脑白质营养不良患者、其杂合子亲属、脑肝肾综合征和新生儿肾上腺脑白质营养不良的生物学识别。
