ALDH5A1 dehydrogenates SUCCSA to SUCCA

Stable Identifier
R-HSA-888548
Type
Reaction [transition]
Species
Homo sapiens
Compartment
Synonyms
Conversion of succinate semialdehyde to succinate
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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).

Literature References
PubMed ID Title Journal Year
19300440 Redox-switch modulation of human SSADH by dynamic catalytic loop

Kim, YG, Lee, S, Kwon, OS, Park, SY, Lee, SJ, Park, BJ, Kim, KJ

EMBO J 2009
9683595 Two exon-skipping mutations as the molecular basis of succinic semialdehyde dehydrogenase deficiency (4-hydroxybutyric aciduria)

Chambliss, KL, Hinson, DD, Trettel, F, Malaspina, P, Novelletto, A, Jakobs, C, Gibson, KM

Am. J. Hum. Genet. 1998
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
succinate-semialdehyde dehydrogenase (NAD+) activity of ALDH5A1 tetramer [mitochondrial matrix]
Physical Entity
Activity
Orthologous Events
Cross References
Rhea
Authored
Reviewed
Created