Definition: two enzymes that are used in the pathway of l-lysine catabolism; the first isoform catalyzes the reversible conversion of l-lysine, a-ketoglutarate, and NADH to saccharopine and NAD+; the other isoform reversibly catalyzes the conversion of saccharopine and NAD+ to l-glutamate, NADH, and l-a-aminoadipate ?-semialdehyde. A deficiency of one of these isoforms is associated with familial hyperlysinemia and saccharopinuria.
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