Katana VentraIP

Succinic semialdehyde

Succinic semialdehyde (SSA) is a GABA and GHB metabolite. It is formed from GABA by the action of GABA transaminase (4-aminobutyrate aminotransferase) and further oxidised to become succinic acid, which enters TCA cycle. SSA is oxidized into succinic acid by the enzyme succinic semialdehyde dehydrogenase, which uses NAD+ as a cofactor.[2] [3]  When the oxidation of succinic semialdehyde to succinic acid is impaired, accumulation of succinic semialdehyde takes place which leads to succinic semialdehyde dehydrogenase deficiency.[3]

In addition to the pathway involving GABA transaminase, gamma-hydroxybutyric acid (GHB) can also be metabolized to SSA via GHB dehydrogenase or by GHB transhydrogenase (D-2-hydroxyglutarate transhydrogenase).[4][5][6][7]

(aminotransferase)

Transaminase

Succinic semialdehyde dehydrogenase deficiency