Profiles in altered metabolism. IV--Induction of acute dicarboxylic aciduria following 2-octynoic acid administration to the rat.
Rats given 2-octynoic acid by intraperitoneal injection excrete elevated amounts of medium-chain dicarboxylic acids and other acidic metabolites usually associated with human medium-chain acyl-CoA dehydrogenase deficiency. Onset of this organic acid profile is immediate and lasts for approximately 24 h. The induced acidosis in this animal model closely, acutely and transiently resembles the human disorder. The 2-octynoate load is also extensively omega- and psi-oxidized, and evidence is presented for the enzymic hydration of 2-octynoate to 3-ketooctanoic acid.
Montgomery
J A
JA
McGill University-Medical Research Council of Canada Biomedical Mass Spectrometry Unit, Montreal, Quebec.
Mamer
O A
OA
Colle
E
E
D000818:Animals; D001786:Blood Glucose / Q000378:metabolism; D002210:Caprylates / Q000378:metabolism; D003998:Dicarboxylic Acids / Q000652:urine*; D005227:Fatty Acids / Q000378:metabolism; D005229:Fatty Acids, Monounsaturated / Q000378:metabolism / Q000494:pharmacology*; D008297:Male; D013058:Mass Spectrometry; D010084:Oxidation-Reduction; D051381:Rats; D011919:Rats, Inbred Strains; D013552:Swine
D001786:Blood Glucose; D002210:Caprylates; D003998:Dicarboxylic Acids; D005227:Fatty Acids; D005229:Fatty Acids, Monounsaturated; C060856:3-ketooctanoic acid; C060855:2-octynoic acid