DETERMINATION OF VERY-LONG-CHAIN FATTY-ACIDS IN PLASMA BY A SIMPLIFIED GAS-CHROMATOGRAPHIC MASS-SPECTROMETRIC PROCEDURE

被引:40
作者
CARUSO, U [1 ]
FOWLER, B [1 ]
ERCEG, M [1 ]
ROMANO, C [1 ]
机构
[1] ROYAL MANCHESTER CHILDRENS HOSP,WILLINK BIOCHEM GENET UNIT,MANCHESTER M27 1HA,LANCS,ENGLAND
来源
JOURNAL OF CHROMATOGRAPHY-BIOMEDICAL APPLICATIONS | 1991年 / 562卷 / 1-2期
关键词
D O I
10.1016/0378-4347(91)80573-U
中图分类号
O65 [分析化学];
学科分类号
070302 ; 081704 ;
摘要
The concentration of very-long-chain fatty acids (VLCFA) (straight chain, more than 22 carbon atoms) in plasma or in cultured fibroblasts is one of the most important diagnostic criteria for the diagnosis of the peroxisomal disorders. A sensitive method for VLCFA assay in plasma, using small sample volume and a simplified procedure, is described. After adequate extraction and derivatization, methyl esters of VLCFA are separated, identificated and quantified by gas chromatography-mass spectrometry (GC-MS). The method is sensitive, reproducible, accurate and relatively simple. GC-MS equipment used for routine organic acid analysis can be used.
引用
收藏
页码:147 / 152
页数:6
相关论文
共 5 条
[1]   SERUM VERY LONG-CHAIN FATTY-ACID PATTERN IN ZELLWEGER SYNDROME [J].
BAKKEREN, JAJM ;
MONNENS, LAH ;
TRIJBELS, JMF ;
MAAS, JM .
CLINICA CHIMICA ACTA, 1984, 138 (03) :325-331
[2]  
Lazarow P. B., 1989, METABOLIC BASIS INHE, P1479
[3]   ADRENOLEUKODYSTROPHY - SURVEY OF 303 CASES - BIOCHEMISTRY, DIAGNOSIS, AND THERAPY [J].
MOSER, HW ;
MOSER, AE ;
SINGH, I ;
ONEILL, BP .
ANNALS OF NEUROLOGY, 1984, 16 (06) :628-641
[4]   ADRENOLEUKODYSTROPHY - INCREASED PLASMA CONTENT OF SATURATED VERY LONG-CHAIN FATTY-ACIDS [J].
MOSER, HW ;
MOSER, AB ;
FRAYER, KK ;
CHEN, W ;
SCHULMAN, JD ;
ONEILL, BP ;
KISHIMOTO, Y .
NEUROLOGY, 1981, 31 (10) :1241-1249
[5]   ADRENOLEUKODYSTROPHY - ELEVATED C26 FATTY-ACID IN CULTURED SKIN FIBROBLASTS [J].
MOSER, HW ;
MOSER, AB ;
KAWAMURA, N ;
MURPHY, J ;
SUZUKI, K ;
SCHAUMBURG, H ;
KISHIMOTO, Y .
ANNALS OF NEUROLOGY, 1980, 7 (06) :542-549