The distal disorders, associated with altered functioning of the cytosolic enzymes, are argininosuccinate synthetase (ASS) deficiency /citrullinemia type I (ASSD; OMIM #215700), argininosuccinate lyase (ASL) deficiency (ASLD; ALD)/argininosuccinic aciduria (ASA; OMIM #207900), and arginase (ARG) deficiency/argininemia (ARGD; OMIM #207800). The gene discussed is ASS1; the disease is hyperinsulinemic hypoglycemia, familial, 4.