Reaction: ALDH5A1 dehydrogenates SUCCSA to SUCCA
- in pathway: Degradation of GABA
Mitochondrial succinate semialdehyde dehydrogenase (ALDH5A1) tetramer catalyzes the reaction of succinate semialdehyde (SUCCSA), H2O, and NAD+ to form succinate (SUCCA) and NADH + 2 H+ (Kim et al. 2009). Mutations that inactivate this gene cause 4-hydroxybutyric aciduria (succinic semialdehyde dehydrogenase deficiency) (Chambliss et al. 1998).
Reaction - small molecule participants:
H+ [mitochondrial matrix]
NADH [mitochondrial matrix]
SUCCA [mitochondrial matrix]
NAD+ [mitochondrial matrix]
SUCCSA [mitochondrial matrix]
H2O [mitochondrial matrix]
Reactome.org reaction link: R-HSA-888548
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Reaction input - small molecules:
NAD(1-)
4-oxobutanoate
water
Reaction output - small molecules:
hydron
NADH(2-)
succinate(2-)
Reactome.org link: R-HSA-888548