4-Guanidinobutanoic acid

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4-Guanidinobutanoic acid
2D structure for 4-Guanidinobutanoic acid
Chemical Name	4-(diaminomethylideneamino)butanoic acid
Chemical Formula	C₅H₁₁N₃O₂
CAS Number	463-00-3
Chemical Information	HMDB03464
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: 0.034 (0.013-0.055) uM Cerebrospinal Fluid (CSF): 0.0125 (0 - 0.025) uM
Normal Tissue Concentrations	Not Available
Diseases / Conditions Related to Nutrition	Cirrhosis
Other (Monogenic Disorders)	Not Available
Abnormal Biofluid Concentrations	Blood (Cirrhosis): 0.05 (0.013-0.09) uM
Abnormal Tissue Concentrations	Not Available
Physiological Processes	Not Available

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Introduction

guidelines
4-Guanidinobutanoate is a normal metabolite present in low concentrations. Patients with hyperargininemia have an arginase deficiency which leads to blockade of the urea cycle in the last step with several clinical symptoms. Owing to the arginase deficiency this patients accumulate arginine which leads eventually to epileptogenic guanidino compounds (PMID 7752905)