Reactome | ALDH5A1 dehydrogenates SUCCSA to SUCCA

ALDH5A1 dehydrogenates SUCCSA to SUCCA

Stable Identifier

R-HSA-888548

Type

Reaction [transition]

Species

Homo sapiens

Compartment

mitochondrial matrix

Synonyms

Conversion of succinate semialdehyde to succinate

ReviewStatus

5/5

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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
9683595	Two exon-skipping mutations as the molecular basis of succinic semialdehyde dehydrogenase deficiency (4-hydroxybutyric aciduria) Chambliss, KL, Gibson, KM, Hinson, DD, Trettel, F, Jakobs, C, Malaspina, P, Novelletto, A	Am. J. Hum. Genet.	1998
19300440	Redox-switch modulation of human SSADH by dynamic catalytic loop Kim, KJ, Park, SY, Lee, S, Kim, YG, Kwon, OS, Park, BJ, Lee, SJ	EMBO J	2009