Determination of Guanidinoacetic Acid in Urine by HPLC-FLD Method
Abstract
Creatine deficiency syndromes are newly recognized inherited metabolic disorders. Creatine is classified into three groups according to the synthesis steps: guanidinoacetate N-methyl transferase (GAMT) deficiency, arginine-glycine amidinotransferase (AGAT) deficiency, and creatine transporter disorder. Clinically, mental retardation, speech delay, epilepsy, movement, and behavioral disorders are prominent. The common biochemical finding in all three diseases is cerebral creatine deficiency. Low urinary guanidinoacetate (GAA) levels are observed in AGAT deficiency and high urinary GAA levels are observed in GAMT deficiency. In this study, we developed a new, simple, inexpensive, and rapid chromatographic analysis method for the quantitative determination of guanidinoacetate in urine samples. Guanidinoacetate was chromatographically separated with a buffer containing 50 mM formic acid and methanol, 1.0 mL/min flow, C18 (150 mm 4.6 mm, 5.0 µm) analytical column and fluorescence detector at ƛex: 390 nm, ƛem: 470 nm.
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References
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