Measurement of very long-chain fatty acids, phytanic and pristanic acid in plasma and cultured fibroblasts by gas chromatography

被引:37
作者
Dacremont, G [1 ]
Cocquyt, G [1 ]
Vincent, G [1 ]
机构
[1] STATE UNIV GHENT HOSP,DEPT PEDIAT,B-9000 GHENT,BELGIUM
关键词
D O I
10.1007/BF00711430
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Two methods are described, both currently used in our laboratory, for the quantitative analysis of very long-chain fatty acids, phytanic acid and pristanic acid in plasma and cultured fibroblasts by gas-liquid chromatography. The first method is based on the procedure developed by Moser and Moser (1991) and the second is based on the method of Onkenhout and colleagues (1989), which is an application of the original method of Lepage and Roy for plasma and fibroblasts. A survey is given of the concentrations of very long-chain fatty acids, pristanic and phytanic acid in plasma and fibroblasts from control subjects and all patients investigated so far in our laboratory.
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页码:76 / 83
页数:8
相关论文
共 9 条
[1]  
LEPAGE G, 1986, J LIPID RES, V27, P114
[2]  
Moser HW, 1991, TECHNIQUES DIAGNOSTI, P177
[3]   IMPROVED DETERMINATION OF VERY-LONG-CHAIN FATTY-ACIDS IN PLASMA AND CULTURED SKIN FIBROBLASTS - APPLICATIONS TO THE DIAGNOSIS OF PEROXISOMAL DISORDERS [J].
ONKENHOUT, W ;
VANDERPOEL, PFH ;
VANDENHEUVEL, MPM .
JOURNAL OF CHROMATOGRAPHY-BIOMEDICAL APPLICATIONS, 1989, 494 :31-41
[4]  
SCHUTGENS RBH, 1993, CLIN CHEM, V39, P1632
[5]   LIGNOCERIC ACID IS OXIDIZED IN THE PEROXISOME - IMPLICATIONS FOR THE ZELLWEGER CEREBRO-HEPATO-RENAL SYNDROME AND ADRENOLEUKODYSTROPHY [J].
SINGH, I ;
MOSER, AE ;
GOLDFISCHER, S ;
MOSER, HW .
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA-BIOLOGICAL SCIENCES, 1984, 81 (13) :4203-4207
[6]  
TENBRINK HJ, 1992, J LIPID RES, V33, P31
[7]  
VANHOVE GF, 1993, J BIOL CHEM, V268, P10335
[8]   POSTNATAL DIAGNOSIS OF PEROXISOMAL DISORDERS - A BIOCHEMICAL APPROACH [J].
WANDERS, RJA ;
SCHUTGENS, RBH ;
BARTH, PG ;
TAGER, JM ;
VANDENBOSCH, H .
BIOCHIMIE, 1993, 75 (3-4) :269-279
[9]   LINKAGE OF DNA MARKERS AT XQ28 TO ADRENOLEUKODYSTROPHY AND ADRENOMYELONEUROPATHY PRESENT WITHIN THE SAME FAMILY [J].
WILLEMS, PJ ;
VITS, L ;
WANDERS, RJA ;
COUCKE, PJ ;
VANDERAUWERA, BJ ;
VANELSEN, AF ;
RAEYMAEKERS, P ;
VAN BROECKHOVEN, C ;
SCHUTGENS, RBH ;
DACREMONT, G ;
LEROY, JG ;
MARTIN, JJ ;
DUMON, JE .
ARCHIVES OF NEUROLOGY, 1990, 47 (06) :665-669