Recent studies from Slc4a11−/− mice have identified glutamine-induced mitochondrial dysfunction as a significant contributor toward oxidative stress, impaired lysosomal function, aberrant autophagy, and cell death in this Congenital Hereditary Endothelial Dystrophy (CHED) model. The gene discussed is SLC4A11; the disease is congenital hereditary endothelial dystrophy of cornea.