Metabolism of deuterium-labeled nonanoic acids in the riboflavin-deficient rat model of multiple acyl-CoA dehydrogenase deficiency.
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 ethyl-malonic, 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 omega-oxidation of nonanoic acids to azelaic acid followed by beta-oxidation to pimelic acid.
Montgomery
J A
JA
McGill University Biomedical Mass Spectrometry Unit, Montreal, Quebec, Canada.
Mamer
O A
OA
Colle
E
E
D044944:Acyl-CoA Dehydrogenases / Q000172:deficiency*; D000818:Animals; D003903:Deuterium; D004195:Disease Models, Animal; D005227:Fatty Acids / Q000378:metabolism*; D008297:Male; D051381:Rats; D011919:Rats, Inbred Strains; D012257:Riboflavin Deficiency / Q000378:metabolism*
D005227:Fatty Acids; C008776:pelargonic acid; D003903:Deuterium; D044944:Acyl-CoA Dehydrogenases