Figure 23.20. Treatment of Carbamoyl Phosphate Synthetase and Ornithine Transcarbamoylase Deficiencies.

Figure 23.20Treatment of Carbamoyl Phosphate Synthetase and Ornithine Transcarbamoylase Deficiencies

Both deficiencies can be treated by supplementing the diet with benzoate and phenylacetate. Nitrogen is excreted in the form of hippurate and phenylacetylglutamine.

From: Section 23.4, Ammonium Ion Is Converted Into Urea in Most Terrestrial Vertebrates

Cover of Biochemistry
Biochemistry. 5th edition.
Berg JM, Tymoczko JL, Stryer L.
New York: W H Freeman; 2002.
Copyright © 2002, W. H. Freeman and Company.

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