saccharopine + NAD+ + H2O => alpha-aminoadipic semialdehyde + glutamate + NADH + H+

Stable Identifier
R-HSA-70940
Type
Reaction [transition]
Species
Homo sapiens
Compartment
Synonyms
AASS tetramer oxidises SACN to 2AMAS
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The saccharopine dehydrogenase activity of lysine-ketoglutarate reductase / saccharopine dehydrogenase homotetramer in the mitochondrial matrix catalyzes the reaction of saccharopine (N6-(L-1,3-Dicarboxypropyl)-L-lysine), H2O, and NAD+ to form 'L-2-Aminoadipate 6-semialdehyde, glutamate, and NADH + H+ (Markovitz et al. 1984; Sacksteder et al. 2001).

Literature References
PubMed ID Title Journal Year
10775527 Identification of the alpha-aminoadipic semialdehyde synthase gene, which is defective in familial hyperlysinemia.

Sacksteder, KA, Biery, BJ, Morrell, JC, Goodman, BK, Geisbrecht, BV, Cox, RP, Gould, SJ, Geraghty, MT

Am J Hum Genet 2001
6434529 Familial hyperlysinemias. Purification and characterization of the bifunctional aminoadipic semialdehyde synthase with lysine-ketoglutarate reductase and saccharopine dehydrogenase activities.

Markovitz, PJ, Chuang, DT, Cox, RP

J Biol Chem 1984
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
saccharopine dehydrogenase (NAD+, L-glutamate-forming) activity of AASS tetramer [mitochondrial matrix]
Physical Entity
Activity
Orthologous Events
Authored