The identification of (E)-2-methylglutaconic acid, a new isoleucine metabolite, in the urine of patients with β-ketothiolase deficiency, propionic acidaemia and methylmalonic acidaemia
✍ Scribed by M. Duran; L. Bruinvis; D. Ketting; J. P. Kamerling; S. K. Wadman; R. B. H. Schutgens
- Publisher
- John Wiley and Sons
- Year
- 1982
- Tongue
- English
- Weight
- 456 KB
- Volume
- 9
- Category
- Article
- ISSN
- 1076-5174
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✦ Synopsis
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 P-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-) methylglutaconic acid ditrimethylsilyl ester are given.