Dosage par chromatographie gazeuse avec détection en photométrie de flamme de l'acide alpha-céto-gammam-méethylthiobutyrique urinaire dans les hyperméthioninémies

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.