Sodium phenylbutyrate (NaPB), a drug commonly employed in the management of urea cycle disorder (UCD), binds to the same allosteric pocket as BT2 to inhibit BCKDK [162], thereby reducing BCAA levels in the plasma of both UCD patients and healthy adults [163,164], as well as the BCAA concentration in culture media observed under experimental conditions [119,165]. This evidence concerns the gene BCKDK and urea cycle disorder.