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The occurrence of β-hydroxy-n-valeric acid in a patient with propionic and methylmalonic acidemia

✍ Scribed by O. Stokke; E. Jellum; L. Eldjarn; R. Schnitler

Book ID
115819738
Publisher
Elsevier Science
Year
1973
Tongue
English
Weight
790 KB
Volume
45
Category
Article
ISSN
0009-8981

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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