Tags: Protein.
L-2-hydroxyglutarate dehydrogenase mitochondrial is an enzyme that in humans is encoded by the L2HGDH gene.This gene encodes L-2-hydroxyglutarate dehydrogenase a FAD-dependent enzyme that oxidizes L-2-hydroxyglutarate to alpha-ketoglutarate in a variety of mammalian tissues. Mutations in this gene cause L-2-hydroxyglutaric aciduria a rare autosomal recessive neurometabolic disorder resulting in moderate to severe mental retardation.