Since genetic disorders causing ADSL deficiency in humans manifest with symptoms affecting the brain, for example, aggressive behavior, autism, intellectual disability, and speech and language impairment, (rarediseases.info.nih.gov), we decided to investigate the metabolic and behavioral effects of the A429V substitution more closely in the mouse as well as the evolution of the ADSL gene in humans. The gene discussed is ADSL; the disease is hyperinsulinemic hypoglycemia, familial, 4.