ALDH4A1 converts 1PYR-3OH-5COOH to 4-OH-L-glutamate
2 H2O + 1PYR-3OH-5COOH + NAD+ → 4OH-L-Glu + H+ + NADH
- Reactome
- R-HSA-6784402
- Compartment
- mitochondrial matrix
- Rhea
- RHEA:31156
Inputs
- 2 H2OCHEBI:15377, H2O, mitochondrial matrix
- 1PYR-3OH-5COOHCHEBI:62612, C5H6NO3, mitochondrial matrix
- NAD+CHEBI:57540, C21H26N7O14P2, mitochondrial matrix
Outputs
- 4OH-L-GluCHEBI:6331, C5H8NO5, mitochondrial matrix
- H+CHEBI:15378, H, mitochondrial matrix
- NADHCHEBI:57945, C21H27N7O14P2, mitochondrial matrix
Catalysed by
- L-glutamate gamma-semialdehyde dehydrogenase activity of ALDH4A1 dimer [mitochondrial matrix]EC 1.2.1.88
Literature
- Hydroxyproline metabolism. IV. Enzymatic synthesis of gamma-hydroxyglutamate from Delta 1-pyrroline-3-hydroxy-5-carboxylate J Biol Chem, 1960
- The three-dimensional structural basis of type II hyperprolinemia J. Mol. Biol., 2012
- Genetic evidence for a common enzyme catalyzing the second step in the degradation of proline and hydroxyproline J Clin Invest, 1979