DLST (dihydrolipoamide S-succinyltransferase) is a protein-coding gene located on chromosome 14 that encodes a mitochondrial protein belonging to the 2-oxoacid dehydrogenase family. This protein is the E2 component of the 2-oxoglutarate dehydrogenase complex, which catalyzes the conversion of 2-oxoglutarate to succinyl-CoA and CO鈧? playing a crucial role in the citric acid cycle. DLST is expressed in various tissues, including the kidney and heart. Mutations in the DLST gene have been associated with diseases such as pheochromocytoma/paraganglioma syndrome.