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Chemical Compound Review:

Carbaglu (2S)-2-(aminocarbonylamino) pentanedioic acid

Synonyms: Carglumic acid, Carbaglu (TN), AG-D-41359, CHEMBL1201780, CHEBI:71028, ...

Caldovic, L. et al., Boyano, M.C. et al., Plecko, B. et al., Frank, J.W. et al.

Frank, Escobar, Nguyen, Jobgen, Jobgen, Davis, Wu,

Welcome! If you are familiar with the subject of this article, you can contribute to this open access knowledge base by deleting incorrect information, restructuring or completely rewriting any text. Read more.

High impact information on L-N-Carbamoylglutamic acid

Oral N-carbamylglutamate supplementation increases protein synthesis in skeletal muscle of piglets [1].
Restoration of ureagenesis in N-acetylglutamate synthase deficiency by N-carbamylglutamate [2].
Treatment with N-carbamyl-L-glutamate (1 mmol/Kg, i.p.) plus L-arginine (1 mmol/kg), which lead to the conversion of ammonia into urea, prevented the ammonium acetate-induced changes in somatostatin binding in this brain area [3].

Associations of L-N-Carbamoylglutamic acid with other chemical compounds

In a patient with N-acetylglutamate synthase (NAGS) deficiency, incorporation of an isotopic label from ammonium chloride into urea was markedly reduced before treatment with N-carbamyl-L-glutamate (NCLG) and completely normalized following treatment [2].

Analytical, diagnostic and therapeutic context of L-N-Carbamoylglutamic acid

CONCLUSION: Partial N-carbamylglutamate deficiency has to be considered in the differential diagnosis of hyperammonaemia [4].

References

Oral N-carbamylglutamate supplementation increases protein synthesis in skeletal muscle of piglets. Frank, J.W., Escobar, J., Nguyen, H.V., Jobgen, S.C., Jobgen, W.S., Davis, T.A., Wu, G. J. Nutr. (2007) [Pubmed]
Restoration of ureagenesis in N-acetylglutamate synthase deficiency by N-carbamylglutamate. Caldovic, L., Morizono, H., Daikhin, Y., Nissim, I., McCarter, R.J., Yudkoff, M., Tuchman, M. J. Pediatr. (2004) [Pubmed]
Somatostatin binding reduced by ammonium acetate in the rat hippocampus can be reversed by treatment with N-carbamyl-L-glutamate plus L-arginine. Boyano, M.C., Bodega, G., Alvaro, I., Arilla, E. Synapse (1992) [Pubmed]
Partial N-acetylglutamate synthetase deficiency in a 13-year-old girl: diagnosis and response to treatment with N-carbamylglutamate. Plecko, B., Erwa, W., Wermuth, B. Eur. J. Pediatr. (1998) [Pubmed]

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