Reaction: ALDH4A1 dimer dehydrogenates 4-OH-L-glutamate semialdehyde to 4-OH-L-glutamate
- in pathway: Glyoxylate metabolism and glycine degradation
Mitochondrial delta-1-pyrroline-5-carboxylate dehydrogenase (ALDH4A1) catalyzes the reaction of 4-hydroxy-L-glutamate gamma-semialdehyde and NAD+ to form 4-hydroxyglutamate and NADH + H+. ALDH4A1 also catalyzes the corresponding reaction of proline catabolism, as shown in biochemical and structural studies (Adams & Goldstone 1960; Srivastava et al. 2012), and mutations in ALDH4A1 disrupt both catabolic processes in human patients (Valle et al. 1979).
Reaction - small molecule participants:
4-OH-L-Glu [mitochondrial matrix]
H+ [mitochondrial matrix]
NADH [mitochondrial matrix]
4-OH-L-Glu semialdehyde [mitochondrial matrix]
NAD+ [mitochondrial matrix]
Reactome.org reaction link: R-HSA-6784399
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Reaction input - small molecules:
L-4-hydroxyglutamate semialdehyde zwitterion
NAD(1-)
Reaction output - small molecules:
4-hydroxy-L-glutamate(1-)
hydron
NADH(2-)
Reactome.org link: R-HSA-6784399