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- Sergej M Ostojic, Laszlo Ratgeber, Andras Olah, Jozsef Betlehem, and Pongras Acs.
- FSPE Applied Bioenergetics Lab, University of Novi Sad, Novi Sad, Serbia.
- Int J Med Sci. 2020 Jan 1; 17 (16): 2544-2550.
AbstractGuanidinoacetic acid (GAA, also known as glycocyamine or betacyamine) is a naturally-occurring derivative of glycine and a direct metabolic precursor of creatine, a key player in high-phosphate cellular bioenergetics. GAA is found in human serum and urine, with circulating GAA likely reflects an equilibrium between its endogenous production and utilization/excretion. GAA deficiency (as indicated by low serum GAA) has been reported in various conditions yet this intriguing clinical entity appears to be poorly characterized as yet, either as a primary deficit or a sequel of secondary disease. This minireview article summarizes the inherited and acquired disorders with apparent GAA deficiency and discusses a possible relevance of GAA shortfall in clinical medicine.© The author(s).
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