Anabolic OTC facilitates the sixth step in the biosynthesis of the amino acid arginine in prokaryotes.

Both binding domains have a similar structural pattern with a central parallel β-pleated sheet bordered by α-helices and loops.

The encoded protein is 354 amino acids long with a predicted molecular weight of 39.935 kD.

Most of the mutations occur in known functional motifs, such as the SMG loop or CP binding domains.

It is classified as a urea cycle disorder due to the fact that without proper OTC function ammonia starts to accumulate in the blood.

Increased levels of orotic acid in urine can be an indicator that a patient is suffering from a disorder linked to hyperammonemia.

The symptoms of a urea cycle disorder are often not seen until the child is at home and may not be recognized in a timely manner by the family and primary care physician.

[17] As ammonia build up continues, symptoms progress from somnolence to lethargy potentially ending in a coma.

Abnormal posturing (uncontrolled movement) and encephalopathy (brain damage) are often related to the degree of central nervous system swelling and pressure upon the brainstem.

Indicators that you maybe suffering from OTC deficiency or a urea cycle disorder include "episodes of delirium, erratic behavior, or reduced consciousness, headaches, vomiting, aversion to foods high in protein, and seizures.

"[18] A potential treatment for the high ammonia levels is to give sodium benzoate, which combines with glycine to produce hippurate, at the same time removing an ammonium group.

Biotin also plays an important role in the functioning of the OTC enzyme[19] and has been shown to reduce ammonia intoxication in animal experiments.