Dysregulation of glutamate dehydrogenase (GDH), an enzyme that catalyzes the oxidative deamination of glutamate, leads to a variety of metabolic and neurological disorders. One such disorder is hyperinsulinism/hyperammonemia syndrome. 1. Describe the role of GDH including the structure, function and biochemistry of the protein and its contribution to the disease. 2. Discuss the several ways that this disease can be diagnosed in the lab Discuss the mechanism by which pharmacological agents act to help in the management of the disease. 3.
