[Determination of urinary alpha-keto-gamma-methylthiobutyric acid in hypermethionemia by use of gas chromatography and flame photometry (author's transl)].
In this paper we describe the determination of urinary alpha-keto-gamma-methylthiobutyric acid (ACMTB) by the use of gas chromatography of its quinoxalinol derivative and specific detection of sulphur with a flame photometric detector. Using these techniques, it was possible to study the urinary elimination of this secondary metabolite of methionine in normal children, homocystinurics and in one case of hypermethioninemia. The excretion of ACMTB is normally very weak and is strongly increased when the principal pathway of methionine is blocked.
D000592:Amino Acid Metabolism, Inborn Errors / Q000097:blood / Q000652:urine*; D002087:Butyrates / Q000652:urine*; D002648:Child; D002849:Chromatography, Gas; D006712:Homocystinuria / Q000652:urine; D006801:Humans; D008715:Methionine / Q000097:blood*; D008722:Methods; D010783:Photometry
D002087:Butyrates; D008715:Methionine
Dosage par chromatographie gazeuse avec détection en photometrie de flamme de l'acide alpha-céto-gamma-méthylthiobutyrique urinaire dans les hyperméthioninémies.