ISSN:
0887-6134
Keywords:
Chemistry
;
Analytical Chemistry and Spectroscopy
Source:
Wiley InterScience Backfile Collection 1832-2000
Topics:
Chemistry and Pharmacology
Notes:
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 Ω- and Ψ-oxidized, and evidence is presented for the enzymic hydration of 2-octynoate to 3-ketooctanoic acid.
Additional Material:
2 Ill.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1002/bms.1200180610