The enzyme deficiencies result in conditions: ornithine transcarbamylase deficiency (OTCD, OMIM #311250), carbamoyl phosphate synthetase I deficiency (CPSD, OMIM #237300), N-acetylglutamate synthase deficiency (NAGS, OMIM #237310), arginosuccinate lyase deficiency (ASL, OMIM #207900), argininosuccinate synthetase deficiency (ASS, OMIM #215700), arginase deficiency (ARG1D, OMIM #207800) [2]. The gene discussed is NAGS; the disease is hyperinsulinemic hypoglycemia, familial, 4.