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

Authors
Libert, R Van Hoof, F Thillaye, M Vincent, MF Nassogne, MC Stroobant, V de Hoffmann, E Schanck, A
Citation
R. Libert et al., Identification of new medium-chain acylcarnitines present in urine of a patient with medium-chain acyl-CoA dehydrogenase deficiency, J INH MET D, 22(1), 1999, pp. 9-18
Citations number
13
Categorie Soggetti
Endocrinology, Nutrition & Metabolism
Journal title
JOURNAL OF INHERITED METABOLIC DISEASE
ISSN journal
01418955 → ACNP
Volume
22
Issue
1
Year of publication
1999
Pages
9 - 18
Database
ISI
SICI code
0141-8955(199902)22:1<9:IONMAP>2.0.ZU;2-K
Abstract
Previously undescribed medium-chain acylcarnitines were identified in a uri ne sample from a patient with medium-chain acyl-CoA dehydrogenase deficienc y. These are the 4-methylvaleryl, 4- and 5-methylhexanoyl, 6-methyl-heptano yl-, 6-methyloctanoyl-, 4,5-dimethylhexanoyl- and 4,7-decadienoylcarnitines . Their chemical structures were obtained by gas chromatography-mass spectr ometry analysis of their fatty acid moieties as picolinyl esters.