AGXT2 (alanine-glyoxylate aminotransferase 2) is a mitochondrial multifunctional aminotransferase with broad substrate specificity that plays crucial roles in cardiovascular and metabolic regulation 1. The enzyme catalyzes multiple reactions including the conversion of glyoxylate to glycine using alanine as amino donor, metabolism of D-beta-aminoisobutyric acid, and transfer of amino groups from beta-alanine to pyruvate 1. Importantly, AGXT2 metabolizes asymmetric (ADMA) and symmetric (SDMA) dimethylarginines, potent inhibitors of nitric oxide synthase, providing a mechanism through which the kidney regulates blood pressure 1. Genetic variants in AGXT2, particularly rs37369 and rs16899974, significantly affect circulating SDMA levels and are associated with cardiovascular disease risk 23. These variants also influence heart rate variability, suggesting AGXT2's role in autonomic nervous system regulation 2. AGXT2 variants have been linked to mild cognitive impairment, with effects mediated through beta-aminoisobutyric acid levels 4. While AGXT2 serves as an alternative pathway for dimethylarginine clearance, evidence suggests it may function primarily as a backup enzyme under pathophysiological conditions rather than being the primary regulator of ADMA/SDMA metabolism 5.