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Metabolism of deuterium‐labeled nonanoic acids in the riboflavin‐deficient rat model of multiple acyl‐CoA dehydrogenase deficiency
Author(s) -
Montgomery Jane A.,
Mamer Orval A.,
Colle Eleanor
Publication year - 1991
Publication title -
biological mass spectrometry
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.475
H-Index - 121
eISSN - 1096-9888
pISSN - 1052-9306
DOI - 10.1002/bms.1200200404
Subject(s) - metabolism , chemistry , riboflavin , biochemistry , deuterium , dehydrogenase , acetyl coa , enzyme , physics , quantum mechanics
Abstract Riboflavin‐deficient rats are used to study the metabolism of deuterium‐labeled nonanoic acids under conditions mimicking the human disorder of multiple acyl‐CoA dehydrogenase deficiency in which large amounts of ethylmalonic, glutaric, adipic, suberic, 4‐octenedioic, sebacic and 4‐decenedioic acids are excreted. Both control and deficient rats convert the nonanoic acids to labeled azelaic and pimelic acids. The labeling pattern in pimelic acid is consistent with the ω‐oxidation of nonanoic acids to azelaic acid followed by β‐oxidation to pimelic acid.