Describe the etiology of urea cycle disorders.

What will be an ideal response?


Ammonia is normally converted to urea in the liver by means of several biochemical steps and eventually excreted as urea. A disruption in any one of the eight component pathways involved in the urea cycle results in disorders of the urea cycle. These pathways are governed by enzymes that undergo a cascade of enzymatic transformations to convert the toxic ammonia molecule to nontoxic, water-soluble urea, which contains two nitrogen groups and is eliminated in the urine. Therefore, urea cycle disorders result in an impaired capacity of the body to excrete nitrogen in the form of urea.

Nutritional Science

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