The dietary management consists of a diet low in native protein, which, on the other hand requires supplementation of essential amino acids in order to meet the requirements.
Urea Cycle Disorders
Urea Cycle Disorders are a series of inherited diseases related to deficiencies of different enzymes involved in the urea cycle. The urea cycle is responsible for detoxification and excretion of ammonia released during protein catabolism, by converting it to non-toxic urea. If not treated, such disorders will lead to accumulation of ammonia and glutamine, which are both neurotoxic.