Tiglicaciduria in propionicacidaemia.
Tiglic acid, which has not previously been found in human body fluids, was recently detected in the urine of two patients with propionicacidaemia. These patients had a documented defect in the oxidation of propionate. A competition between acrylyl-CoA and tiglyl-CoA for crotonase could explain the accumulation of tiglic acid.
D000179:Acrylates / Q000378:metabolism; D002087:Butyrates / Q000652:urine*; D002247:Carbon Isotopes; D002849:Chromatography, Gas; D003065:Coenzyme A / Q000378:metabolism; D006801:Humans; D006836:Hydro-Lyases / Q000378:metabolism; D013058:Mass Spectrometry; D008661:Metabolism, Inborn Errors / Q000652:urine*; D010084:Oxidation-Reduction; D011422:Propionates / Q000097:blood*
D000179:Acrylates; D002087:Butyrates; D002247:Carbon Isotopes; D011422:Propionates; D006836:Hydro-Lyases; D003065:Coenzyme A