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DraconiticBiochemistryBiomolecules · Catabolism · Biosynthesis · MetabolismMap
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ALDH4A1 oxidises L-GluSS to Glu

L-Glu5S + NAD+ + H2O → L-Glu + NADH + 2 H+

Reactome
R-HSA-70679
Compartment
mitochondrial matrix
Rhea
RHEA:30236

Inputs

Outputs

Catalysed by

  • L-glutamate gamma-semialdehyde dehydrogenase activity of ALDH4A1 dimer [mitochondrial matrix]EC 1.2.1.88

Literature

  1. Purification and characterization of human liver "high Km" aldehyde dehydrogenase and its identification as glutamic gamma-semialdehyde dehydrogenase. J Biol Chem, 1986
  2. Mutations in the Delta1-pyrroline 5-carboxylate dehydrogenase gene cause type II hyperprolinemia. Hum Mol Genet, 1998