N-Acetylglutamate synthetase: enzyme assay in human liver.
In the context of diagnostic procedures for congenital hyperammonaemias a methods is described for the determination of N-acetylglutamate synthetase in human liver tissue homogenates. The method uses [14C-U] glutamate and acetyl CoA as substrates. The reaction product, N-acetylglutamate is separated from the substrate L-glutamate by chromatography on Extrelut. In a subsequent step on ITLC-SG ready plates N-acetylglutamate is separated from other labeled metabolites such as Krebs cycle intermediates. The recovery of N-acetylglutamate was 97.8%. The precision within run and between days was 8.5% (CV) and 9.6% (CV) respectively. Reference values were established for adult human liver.
D000123:Acetyltransferases / Q000032:analysis*; D051046:Amino-Acid N-Acetyltransferase; D001120:Arginine; D055598:Chemical Phenomena; D002621:Chemistry; D002845:Chromatography / Q000379:methods; D002855:Chromatography, Thin Layer / Q000379:methods; D005971:Glutamates; D006801:Humans; D006863:Hydrogen-Ion Concentration; D007700:Kinetics; D008099:Liver / Q000201:enzymology*; D013379:Substrate Specificity
D005971:Glutamates; D001120:Arginine; D000123:Acetyltransferases; D051046:Amino-Acid N-Acetyltransferase; C494874:NAGS protein, human