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Deranged isoleucine metabolism during ketotic attacks in patients with methylmalonic acidaemia

✍ Scribed by M. Duran; L. Bruinvis; D. Ketting; S. K. Wadman


Publisher
Springer
Year
1978
Tongue
English
Weight
398 KB
Volume
1
Category
Article
ISSN
0141-8955

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The identification of (E)-2-methylglutac
✍ 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