Title : Molecular characterization of carbamoyl-phosphate synthetase (CPS1) deficiency using human recombinant CPS1 as a key tool.

Pub. Date : 2013 Aug

PMID : 23649895






1 Functional Relationships(s)
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1 NAG and its analogue and orphan drug N-carbamoyl-l-glutamate, protected human CPS1 against proteolytic and thermal inactivation in the presence of MgATP, raising hopes of treating CPS1D by chemical chaperoning with N-carbamoyl-l-glutamate. N-carbamylglutamate N-acetyl-alpha-glucosaminidase Homo sapiens