Dihydrolipoyllysine-residue succinyltransferase

• OMIM: 126063
• UniProt: P36957

The Dihydrolipoyl transsuccinylase ( KDH -E2 ) (exactly: Dihydrolipoyllysinrest succinyltransferase ) is an enzyme and one of the three components of the ketoglutarate dehydrogenase enzyme complex which is indispensable in the Krebs cycle and in the lysine degradation of eukaryotes and some bacteria. KDH -E2 catalyzes the transfer of Succinylrests of the to the enzyme belonging Dihydrolipoyllysin -amino acid residue to a coenzyme A molecule. It thus acts similar to the dihydrolipoyl transacetylase the pyruvate dehydrogenase complex ( PDH E2), but does not have binding domains for the E1 and E3 - unit. KDH E2 forms 24 -mers with octahedral symmetry.

A lower production of KDH -E2 in old age or by mutation may be the cause of neurodegenerative diseases.