The ETFDH gene encodes an enzyme called electron transfer flavoprotein dehydrogenase. This enzyme is active in the inner mitochondrial membrane, where it accepts electrons from electron-transfer flavoprotein located in the mitochondrial matrix and reduces ubiquinone in the mitochondrial membrane. Defects in this enzyme are the cause of glutaric aciduria 2C (GA2C), an autosomal recessive disorder characterized by multiple acyl-CoA dehydrogenase deficiencies resulting in large excretion not only of glutaric acid, but also of lactic, ethylmalonic, butyric, isobutyric, 2-methyl-butyric, and isovaleric acids.