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).
Chambliss, KL, Gibson, KM, Hinson, DD, Trettel, F, Jakobs, C, Malaspina, P, Novelletto, A
Kim, KJ, Park, SY, Lee, S, Kim, YG, Kwon, OS, Park, BJ, Lee, SJ
succinate-semialdehyde dehydrogenase (NAD+) activity of ALDH5A1 tetramer [mitochondrial matrix]
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