Online edition:ISSN 2758-089X


Very Long Chain Fatty Acid Contents in the Sera of Patients with Adrenoleukodystrophy and Adrenomyeloneuropathy Measured by Selected-Ion-Monitoring Gas Chromatography/Mass Spectrometry

Saturated very long chain fatty acid (VLCFAs, C22:0 - C28 :0) contents in the sera of 9 adrenoleukodystrophy (ALD) patients, 4 adrenomyeloneuropathy (AMN) patients, and 11 age-matched normal controls were determined by gas chromatography (GC), GC/mass spectrometry (MS) and selected-ion-monitoring (SIM)-GC/MS. The following results were obtained. The C22:0 contents of the ALD and AMN groups were not significantly different from those of the control group. However, the C24: 0 contents of the ALD and AMN groups were respectively, 1.9 and 1.5 times higher than control, and the C26:0 contents of these groups measured by SIM-GC/MS were 7.7 and 7.8 times higher than the control. These contents were about half of those measured by GC alone. The C28 :0 content of human sera could be measured for the first time in this study using SIM-GC/MS. The order of the increasing rate of the individual VLCFAs of ALD and AMN as compared that with the control was as follows, C26 : 0 > C28 : 0 > C24 : 0.

Kawai K.