tiglyl-coenzyme-a has been researched along with tiglylglycine* in 1 studies
1 other study(ies) available for tiglyl-coenzyme-a and tiglylglycine
Article | Year |
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The identification of (E)-2-methylglutaconic acid, a new isoleucine metabolite, in the urine of patients with beta-ketothiolase deficiency, propionic acidaemia and methylmalonic acidaemia.
The identification of (E)-2-methylglutaconic acid, a 'new' metabolite of isoleucine, is described. The substance was detected in urine samples from patients with propionic acidaemia, methylmalonic acidaemia and so-called beta-ketothiolase deficiency; in the majority of cases together with N-tiglylglycine. (E)-2-Methylglutaconic acid is thought to be the end product of the 3-methylcrotonyl-CoA carboxylase-catalysed carboxylation of tiglyl-CoA. Prerequisites for the quantitative gas chromatographic analysis of the unstable 2- (and 3-) methyl-glutaconic acid ditrimethylsilyl ester are given. Topics: Acetyl-CoA C-Acyltransferase; Acyl Coenzyme A; Acyltransferases; Amino Acid Metabolism, Inborn Errors; Gas Chromatography-Mass Spectrometry; Glutarates; Glycine; Humans; Isoleucine; Malonates; Methylmalonic Acid; Propionates; Reference Values | 1982 |