saccharopine + NAD+ + H2O => alpha-aminoadipic semialdehyde + glutamate + NADH + H+
SACN + NAD+ + H2O → 2AMAS + L-Glu + NADH + H+
- Reactome
- R-HSA-70940
- Compartment
- mitochondrial matrix
- Rhea
- RHEA:24521
Inputs
- SACNCHEBI:57951, C11H19N2O6, mitochondrial matrix
- NAD+CHEBI:57540, C21H26N7O14P2, mitochondrial matrix
- H2OCHEBI:15377, H2O, mitochondrial matrix
Outputs
- 2AMASCHEBI:58321, C6H11NO3, mitochondrial matrix
- L-GluCHEBI:29985, C5H8NO4, mitochondrial matrix
- NADHCHEBI:57945, C21H27N7O14P2, mitochondrial matrix
- H+CHEBI:15378, H, mitochondrial matrix
Catalysed by
- saccharopine dehydrogenase (NAD+, L-glutamate-forming) activity of AASS tetramer [mitochondrial matrix]EC 1.5.1.9
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