3-Methylcrotonylglycine excretion in 3-hydroxy-3-methylglutaric aciduria

3-Methylcrotonylglycine excretion in 3-hydroxy-3-methylglutaric aciduria

1. 1. 3-Methylcrotonylglycine was identified in urine from an infant with 3-hydroxy-3-methylglutaric aciduria. 2. 2. The concentration of 3-methylcrotonylglycine in urine was approximately one sixth of that of the other metabolite of 3-methylcrotonyl-CoA, 3-hydroxyisovaleric acid. 3. 3. The presence...

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Veröffentlicht in:Clinica chimica acta 1978-05, Vol.86 (1), p.101-108
Hauptverfasser: Wysocki, S.J., Hähnel, R.
Format: Artikel
Sprache:eng
Schlagworte:
Crotonates - urine
Glutarates - urine
Glycine - analogs & derivatives
Glycine - chemical synthesis
Glycine - urine
Humans
Hydroxy Acids - urine
Infant
Male
Metabolism, Inborn Errors - urine
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Zusammenfassung:1. 1. 3-Methylcrotonylglycine was identified in urine from an infant with 3-hydroxy-3-methylglutaric aciduria. 2. 2. The concentration of 3-methylcrotonylglycine in urine was approximately one sixth of that of the other metabolite of 3-methylcrotonyl-CoA, 3-hydroxyisovaleric acid. 3. 3. The presence of both metabolites in the infant's urine indicates an inhibition of 3-methylcrotonyl-CoA carboxylase activity in tissues of the infant.
ISSN:0009-8981
1873-3492
DOI:10.1016/0009-8981(78)90464-3