Reaction: ALDH4A1 oxidises L-GluSS to Glu
- in pathway: Proline catabolism
Mitochondrial delta-1-pyrroline-5-carboxylate dehydrogenase (ALDH4A1) catalyzes the reaction of L-glutamate gamma-semialdehyde and NAD+ to form glutamate and NADH + H+ (Hu et al. 1996). The enzyme is a dimer (Forte-McRobbie and Pietruszko 1986). ALDH4A1 mutations cause type II hyperprolinemia in vivo (Geraghty et al. 1998).
Reaction - small molecule participants:
H+ [mitochondrial matrix]
NADH [mitochondrial matrix]
L-Glu [mitochondrial matrix]
L-Glu5S [mitochondrial matrix]
NAD+ [mitochondrial matrix]
H2O [mitochondrial matrix]
Reactome.org reaction link: R-HSA-70679
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Reaction input - small molecules:
L-glutamic 5-semialdehyde zwitterion
NAD(1-)
water
Reaction output - small molecules:
hydron
NADH(2-)
L-glutamate(1-)
Reactome.org link: R-HSA-70679