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

Stable Identifier
R-HSA-70938
Type
Reaction [transition]
Species
Homo sapiens
Compartment
Locations in the PathwayBrowser
General
SVG |   | PPTX  | SBGN
Click the image above or here to open this reaction in the Pathway Browser
The layout of this reaction may differ from that in the pathway view due to the constraints in pathway layout

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
Participants
Participates
Catalyst Activity

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

Orthologous Events
Cross References
Rhea
Authored
Cite Us!