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Identification of 3-hydroxy-3-ethylglutaric acid in urine of patients with propionic acidaemia

✍ Scribed by Tomiko Kuhara; Yoshito Inoue; Toshihiro Shinka; Isamuu Matsumoto; Masafumi Matsuo

Publisher
John Wiley and Sons
Year
1983
Tongue
English
Weight
360 KB
Volume
10
Category
Article
ISSN
1076-5174

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The identification of (E)-2-methylglutac
The identification of (E)-2-methylglutaconic acid, a new isoleucine metabolite, in the urine of patients with Ξ²-ketothiolase deficiency, propionic acidaemia and methylmalonic acidaemia
✍ M. Duran; L. Bruinvis; D. Ketting; J. P. Kamerling; S. K. Wadman; R. B. H. Schut πŸ“‚ Article πŸ“… 1982 πŸ› John Wiley and Sons 🌐 English βš– 456 KB πŸ‘ 1 views

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-tiglylglyci