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 utilizes electron transfer flavoprotein as an electron acceptor and forms a homotetramer of 45-kD subunits within the mitochondrial matrix. Mutations in this gene cause glutaric aciduria type 1, also known as glutaric acidemia type I.