Kinetic analysis of argininosuccinate synthetase in a variant form of citrullinaemia

Argininosuccinate (ASA) synthetase in the liver from a patient with a variant form of citrullinaemia was analysed. Serum citrulline level was approximately 40 times higher than the control level, and the ASA synthetase activity was approximately 10% that of the control. Other urea cycle enzymes were...

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Veröffentlicht in:	Journal of inherited metabolic disease 1983-03, Vol.6 (1), p.36-39
Hauptverfasser:	Akaboshi, I., Endo, F., Matsuda, I., Saheki, T.
Format:	Artikel
Sprache:	eng
Schlagworte:	Adult Amino Acid Metabolism, Inborn Errors - enzymology Argininosuccinate Synthase - analysis argininosuccinate synthetase Citrulline - blood citrullinemia Humans Hydrogen-Ion Concentration Kinetics Ligases - analysis liver Liver - enzymology Male man
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