Accession NumberHMDB04225
Common_Name2-Oxoarginine
Description2-Oxoarginine is a guanidino compound metabolite of arginine catabolism. 2-Oxoarginine levels are increased in patients with Argininemia (OMIM 207800). Argininemia, characterized by arginase deficiency (EC 3.5.3.1, catalyzes the last step of the urea cycle) is an autosomal recessive inborn error of metabolism caused by a defect in the final step in the urea cycle, the hydrolysis of arginine to urea and ornithine. Accumulation of arginine metabolites (such as guanidino compounds) especially 2-oxoarginine, may produce the central nervous system damage in argininemia. (PMID: 3433275, 1588833, 1690873, 819629)
Chemical_IUPAC_Name5-(diaminomethylideneamino)-2-oxo-pentanoic acid
Chemical FormulaC6H11N3O3
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