|
Medical Definition of Saccharopine dehydrogenase
1. Two enzymes that are used in the pathway of l-lysine catabolism; the first isoform catalyses the reversible conversion of l-lysine, alpha-ketoglutarate, and NADH to saccharopine and NAD+; the other isoform reversibly catalyses to conversion of saccharopine and NAD+ to l-glutamate, NADH, and l-alpha-aminoadipate d-semialdehyde. A deficiency of one of these isoforms is associated with familial hyperlysinaemia and saccharopinuria. (05 Mar 2000)