Saccharopine dehydrogenase (SCCPDH)

Saccharopine dehydrogenase (SCCPDH) is an enzyme involved in the degradation of L-lysine via the saccharopine pathway, catalyzing the oxidative deamination of saccharopine to produce L-glutamate and 2-aminoadipate 6-semialdehyde with reduction of NAD+ to NADH[2][3][5][6][7]. The gene SCCPDH encodes a putative member of the saccharopine dehydrogenase family and is predicted to possess oxidoreductase activity, but its exact biological roles in humans are incompletely understood[2][4][6][7]. The enzyme’s deficiency is implicated in metabolic disorders such as familial hyperlysinemia, which is characterized by increased levels of lysine and saccharopine and can result in neurological symptoms[5]. Multiple transcript aliases and synonyms refer to this protein across different databases and species[2][4][6][7][8]. In mammals, the enzyme participates in nitrogen balance and amino acid catabolism, with expression reported in the liver, kidney, and possibly other tissues[5]. No approved drugs are known to directly target SCCPDH, nor are there established clinical mechanisms of action or development programs; its role as a clinical target is defined primarily by its pathophysiological involvement in rare metabolic diseases[5][7]. There are no currently established safety concerns for drug development beyond the consequences of loss-of-function mutations and the resulting metabolic imbalance.

Other names

saccharopine dehydrogenase-like oxidoreductaseCGI-49NET11FLJ43187RP11-439E19.2LOC103689999epididymis secretory sperm binding proteinprobable saccharopine dehydrogenaseSCPDL