2-Oxoarginine

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2-Oxoarginine
2D structure for 2-Oxoarginine
Chemical Name	5-(diaminomethylideneamino)-2-oxo-pentanoic acid
Chemical Formula	C₆H₁₁N₃O₃
CAS Number	3715-10-4
Chemical Information	HMDB04225
Biochemical Taxonomy	Amino Acids
Functional Taxonomy	Not Available
Nutritional Taxonomy	Not Available
Metabolic Pathways	Not Available
Biofluid Location	Blood Cerebrospinal Fluid (CSF)
Tissue Location	Not Available
Normal Biofluid Concentrations	Blood: 0.145 +/- 0.007 umol/L Cerebrospinal Fluid (CSF): 0.12 +/- 0.12 (0- 0.25) uM
Normal Tissue Concentrations	Not Available
Diseases / Conditions Related to Nutrition	Patients with Hypothyroidism Patients with hyperthyroidism
Other (Monogenic Disorders)	Not Available
Abnormal Biofluid Concentrations	Blood (Patients with Hypothyroidism): 0.134 +/- 0.014 umol/L Blood (Patients with hyperthyroidism): 0.160 +/- 0.009 umol/L
Abnormal Tissue Concentrations	Not Available
Physiological Processes	Not Available

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Introduction

guidelines
2-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)