The identification of 3-keto-2-methylvaleric acid and 3-hydroxy-2-methylvaleric acid in a patient with propionic acidemia
β Scribed by Roger J. W. Truscott; Christopher J. Pullin; Berthold Halpern; Judy Hammond; Eric Haan; David M. Danks
- Publisher
- John Wiley and Sons
- Year
- 1979
- Tongue
- English
- Weight
- 702 KB
- Volume
- 6
- Category
- Article
- ISSN
- 1076-5174
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## Abstract For Abstract see ChemInform Abstract in Full Text.
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
Iicccivcd Fcbrunry x 0th) 1 gGg) EXPERIMENTAL Reagents z,z-Diethylpropanediol-I,3 (DEPD; J. T. Baker Chemical Co, Phillipsburg, U.S.A.) and 2-ethylhexanediol-1,s (EHD; Kebo AB, Stockholm, Sweden) were purified as in the previous workis. Chloroform (p.a.) was washed twice with redistilled water and t