lysine + alpha-ketoglutarate +NADPH + H+ => saccharopine + NADP+ + H2O
L-Lys + 2OG + NADPH + H+ → SACN + NADP+ + H2O
- Reactome
- R-HSA-70938
- Compartment
- mitochondrial matrix
- Rhea
- RHEA:19375
Inputs
- L-LysCHEBI:32551, C6H15N2O2, mitochondrial matrix
- 2OGCHEBI:16810, C5H4O5, mitochondrial matrix
- NADPHCHEBI:57783, C21H26N7O17P3, mitochondrial matrix
- H+CHEBI:15378, H, mitochondrial matrix
Outputs
- SACNCHEBI:57951, C11H19N2O6, mitochondrial matrix
- NADP+CHEBI:58349, C21H25N7O17P3, mitochondrial matrix
- H2OCHEBI:15377, H2O, mitochondrial matrix
Catalysed by
- saccharopine dehydrogenase (NADP+, L-lysine-forming) activity of AASS tetramer [mitochondrial matrix]EC 1.5.1.8
Literature
- Familial hyperlysinemias. Purification and characterization of the bifunctional aminoadipic semialdehyde synthase with lysine-ketoglutarate reductase and saccharopine dehydrogenase activities. J Biol Chem, 1984
- Identification of the alpha-aminoadipic semialdehyde synthase gene, which is defective in familial hyperlysinemia. Am J Hum Genet, 2001