lysine + alpha-ketoglutarate +NADPH + H+ => saccharopine + NADP+ + H2O

Stable Identifier
Reaction [transition]
Homo sapiens
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The saccharopine dehydrogenase activity of lysine-ketoglutarate reductase / saccharopine dehydrogenase homotetramer in the mitochondrial matrix catalyzes the reaction of lysine, alpha-ketoglutarate, and NADPH + H+ to form saccharopine, NADP+, and H2O (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.

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

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
Catalyst Activity

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

Orthologous Events
Cross References
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