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Results 11 - 14 of 14

EC Number	Natural Substrates	Commentary (Nat. Sub.)
2.3.1.1	more	autosomal recessively inherited enzyme deficiency causes severe neonatal or late-onset hyperammonemia, the enzyme is an allosteric activator of the carbamoylphosphate synthase I, the first enzyme of the urea cycle
2.3.1.1	more	enzyme deficiency causes hyperammonemia, presumably due to loss of carbamoylphosphate synthase I activity
2.3.1.1	more	the bifunctional N-acetyl-L-glutamate synthase/kinase contains an N-terminal catalytic N-acetyltransferase (NAT) domain, substrate N-acetyl-l-glutamate binding structure analysis, overview
2.3.1.1	more	the N-acetyl-L-glutamate synthase contains an N-terminal catalytic N-acetyltransferase domain and a C-terminal amino acid kinase domain

Results 11 - 14 of 14

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Release 2023.1 (January 2023)