Such elevated neuronal glutamine metabolism has been demonstrated in models of Alzheimer's disease (Andersen, Christensen, et al. 2021), SSADH deficiency (Andersen et al. 2024) and frontotemporal dementia type 3 (Aldana et al. 2020), further underlining the general importance of glutamine as a metabolic substrate in neurons. The gene discussed is ALDH5A1; the disease is hyperinsulinemic hypoglycemia, familial, 4.