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

Stable Identifier
R-HSA-70940
Type
Reaction [transition]
Species
Homo sapiens
Compartment
Synonyms
H2O + L-saccharopine + NAD(+) => (S)-2-amino-6-oxohexanoate + H(+) + L-glutamate + NADH, AASS tetramer oxidises SACN to 2AMAS
ReviewStatus
5/5
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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
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
10775527 Identification of the alpha-aminoadipic semialdehyde synthase gene, which is defective in familial hyperlysinemia.

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

Am J Hum Genet 2001
Participants
Participates
Catalyst Activity

saccharopine dehydrogenase (NAD+, L-glutamate-forming) activity of AASS tetramer [mitochondrial matrix]

Orthologous Events
Cross References
RHEA
Rhea
Authored
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