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Identification of new medium‐chain acylcarnitines present in urine of a patient with medium‐chain acyl‐CoA dehydrogenase deficiency
Author(s) -
Libert R.,
Van Hoof F.,
Thillaye M.,
Vincent M.F.,
Nassogne M.C.,
Stroobant V.,
Hoffmann E.,
Schanck A.
Publication year - 1999
Publication title -
journal of inherited metabolic disease
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.462
H-Index - 102
eISSN - 1573-2665
pISSN - 0141-8955
DOI - 10.1023/a:1005482828392
Subject(s) - urine , chemistry , dehydrogenase , acyl coa dehydrogenase , biochemistry , lactate dehydrogenase , enzyme , chain (unit) , chromatography , physics , astronomy
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‐decadienoyl‐carnitines. Their chemical structures were obtained by gas chromatography–mass spectrometry analysis of their fatty acid moieties as picolinyl esters.