The elimination of waste nitrogen as urea is a final and central step of amino acid catabolism. It is accomplished by one of the most essential pathways of terrestrial animals, the urea cycle. This chapter describes the basic function of the 5 catalytic enzymes, the two transporters, and the cofactor synthesizing enzyme that comprise the urea cycle as well as the consequences of their deficiencies and ways to treat them. The chapter then elaborates on related disorders of metabolism that may either cause hyperammonemia or elevation of an amino acid that is key to urea cycle function.
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