Urea cycle function, primary

Although the primary function of the urea cycle is usually regarded as the removal of NH4+ from the body, it also removes HC03 in equal amounts (Eq. 24-24). This is essential for maintenance of neutral pH,... 

A urea cycle disorder is caused by a deficiency in any one of six enzymes in the urea cycle [1] (Fig. 15.1). Collectively, urea cycle disorders (UCD) are relatively common, with an incidence of 1 35,000 births [2]. The primary function of the urea cycle is to remove nitrogen produced... 

The primary function of the urea cycle is to rid the body of waste nitrogen. Deficiency in the activity of any of the six enzymes in the urea cycle may result in the accumulation of ammonia, often to toxic concentrations. Treatment involves restricting protein, preventing catabolism, supplementing amino acids that are normally produced by the urea cycle, and promoting the excretion of nitrogen via alternative pathways. Outcomes are guarded and appear to be better for patients identified by NBS compared to patients identified clinically. Liver transplantation is a treatment option, especially for patients with a severe form of the disorder. 

---