Identification of new medium-chain acylcarnitines present in urine of a patient with medium-chain acyl-CoA dehydrogenase deficiency

被引:9
作者
Libert, R
Van Hoof, F
Thillaye, M
Vincent, MF
Nassogne, MC
Stroobant, V
de Hoffmann, E
Schanck, A
机构
[1] Clin Univ St Luc, Dept Biol Clin, Malad Metab Lab, B-1200 Brussels, Belgium
[2] Univ Catholique Louvain, Dept Chim, Lab Spectrometrie Masse, B-1348 Louvain, Belgium
[3] Univ Catholique Louvain, Dept Chim, Chim Phys & Cristallog Lab, B-1348 Louvain, Belgium
[4] Univ Catholique Louvain, Dept Chim, Ctr Adv Mat Res, B-1348 Louvain, Belgium
关键词
D O I
10.1023/A:1005482828392
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Previously undescribed medium-chain acylcarnitines were identified in a urine sample from a patient with medium-chain acyl-CoA dehydrogenase deficiency. These are the 4-methylvaleryl, 4- and 5-methylhexanoyl, 6-methyl-heptanoyl-, 6-methyloctanoyl-, 4,5-dimethylhexanoyl- and 4,7-decadienoylcarnitines. Their chemical structures were obtained by gas chromatography-mass spectrometry analysis of their fatty acid moieties as picolinyl esters.
引用
收藏
页码:9 / 18
页数:10
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