Argininic acid

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Argininic acid
2D structure for Argininic acid
Chemical Name	(2S)-5-(diaminomethylideneamino)-2-hydroxy-pentanoic acid
Chemical Formula	C₆H₁₃N₃O₃
CAS Number	157-07-3
Chemical Information	HMDB03148
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: 0.2 (0.015-0.44) uM
Normal Tissue Concentrations	Not Available
Diseases / Conditions Related to Nutrition	Cirrhotic patients Hyperargininemia
Other (Monogenic Disorders)	Not Available
Abnormal Biofluid Concentrations	Blood (Cirrhotic patients): 0.25 (0.015-0.5) uM Cerebrospinal Fluid (CSF) (Hyperargininemia): uM
Abnormal Tissue Concentrations	Not Available
Physiological Processes	Not Available

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Introduction

guidelines
Tissue accumulation of argininic acid (AA) occurs in hyperargininemia. Hyperargininemia, an inborn error of the urea cycle, is caused by a severe deficiency of liver arginase, resulting in elevated tissue levels of arginine (Arg) and other guanidino compounds (GC). Affected patients present a neurological syndrome consisting of a variable degree of mental retardation, epilepsy and progressive spasticity whose pathophysiology is far from understood. Guanidino compounds accumulate in other pathological conditions such as uremia and epilepsy and some evidence supports the hypothesis that these compounds contribute to the neurological dysfunction characteristic of these diseases. The increase of these compounds occurs by blockage of the arginase reaction, activating secondary biochemical pathways. Thus, Arg is converted to α-keto-δ-guanidinovaleric acid by transamination, and this compound forms AA by hydrogenation. (PMID 12615083)