| Summary: | 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.
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