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

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

Stable Identifier

R-HSA-70940

Type

Reaction [transition]

Species

Homo sapiens

Compartment

mitochondrial matrix

Synonyms

AASS tetramer oxidises SACN to 2AMAS

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

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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