It uses electron transfer flavoprotein as its electron acceptor. The enzyme exists in the mitochondrial matrix as a homotetramer of 45-kD subunits. Mutations in this gene result in the metabolic disorder glutaric aciduria type 1, which is also known as glutaric acidemia type I.
The enzyme relies on electron transfer flavoprotein for electron acceptor function and is a homotetramer of 45-kD subunits within the mitochondrial matrix. Genetic variations in this gene can result in glutaric aciduria type 1, also referred to as glutaric acidemia type I.