title: New pathways of nitrogen excretion in inborn errors of urea synthesis.
abstract: The defect in nitrogen excretion in patients with inborn errors of urea synthesis can be controlled by exploiting the biosynthetic pathways of readily excretable non-urea metabolites which contain nitrogen derived from ammonium, alanine, glutamate, and glutamine. Two classes of such metabolites are the urea-cycle intermediates--including citrulline, argininosuccinic acid, and arginine--and the aminoacid acylation products--hippuric acid (the glycine conjugate of benzoic acid) and phenylactylglutamine (the glutamine conjugate of phenylactic acid). Thus the urea cycle may serve as a model for the development of excretion pathways of toxic precursors which accumulate in inborn errors of metabolism.
mesh_terms: D001120:Arginine / Q000627:therapeutic use; D001124:Argininosuccinate Synthase / Q000172:deficiency; D056807:Argininosuccinic Aciduria; D002956:Citrulline / Q000627:therapeutic use; D004044:Dietary Proteins / Q000008:administration & dosage; D006626:Hippurates / Q000627:therapeutic use; D006801:Humans; D007223:Infant; D008661:Metabolism, Inborn Errors / Q000378:metabolism / Q000628:therapy*; D009584:Nitrogen / Q000652:urine*; D020163:Ornithine Carbamoyltransferase Deficiency Disease; D009963:Orotic Acid / Q000627:therapeutic use; D010648:Phenylacetates / Q000627:therapeutic use; D014508:Urea / Q000096:biosynthesis*
chemical_list: D004044:Dietary Proteins; D006626:Hippurates; D010648:Phenylacetates; D002956:Citrulline; D009963:Orotic Acid; D014508:Urea; D001120:Arginine; D001124:Argininosuccinate Synthase; D009584:Nitrogen