Article
Structure of human carbamoyl phosphate synthetase: deciphering the on/off switch of human ureagenesis.
Scientific reports - 23 Nov 2015
de Cima Sergio, Polo Luis M, Díez-Fernández Carmen, Martínez Ana I, Cervera Javier, Fita Ignacio, Rubio Vicente
Abstract excerpt
Human carbamoyl phosphate synthetase (CPS1), a 1500-residue multidomain enzyme, catalyzes the first step of ammonia detoxification to urea requiring N-acetyl-L-glutamate (NAG) as essential activator to prevent ammonia/amino acids depletion. Here we present the crystal structures of CPS1 in the absence and in the presence of NAG, clarifying the on/off-switching of the urea cycle by NAG. By binding at the...
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