Succinate-semialdehyde dehydrogenase (SSADH)

Succinate-semialdehyde dehydrogenase (SSADH) is an enzyme crucial for the degradation of γ-aminobutyric acid (GABA), a major inhibitory neurotransmitter in the central nervous system. The enzyme catalyzes the conversion of succinate semialdehyde to succinic acid, using NAD+ as a cofactor. This reaction is part of the GABA shunt, linking neurotransmitter metabolism to cellular energy production via the tricarboxylic acid (TCA) cycle. Deficiency or mutations in ALDH5A1 cause succinic semialdehyde dehydrogenase deficiency (also called SSADHD or gamma-hydroxybutyric aciduria), an autosomal recessive disorder characterized by neurological symptoms and elevated levels of GHB/GABA.

Other names

succinic semialdehyde dehydrogenasesuccinyl semialdehyde dehydrogenase

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