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Names | |
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Preferred IUPAC name
4-Oxobutanoic acid | |
Identifiers | |
3D model (
JSmol)
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|
ChEBI | |
ChemSpider | |
ECHA InfoCard | 100.155.728 |
KEGG | |
MeSH | Succinic+semialdehyde |
PubChem
CID
|
|
UNII | |
CompTox Dashboard (
EPA)
|
|
| |
| |
Properties | |
C4H6O3 | |
Molar mass | 102.089 g/mol |
Appearance | oil |
Boiling point | 135 °C (275 °F; 408 K) at 14 mmHg |
soluble in water, ethanol, benzene, diethyl ether [1] | |
Except where otherwise noted, data are given for materials in their
standard state (at 25 °C [77 °F], 100 kPa).
|
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]
![]() | |
Names | |
---|---|
Preferred IUPAC name
4-Oxobutanoic acid | |
Identifiers | |
3D model (
JSmol)
|
|
ChEBI | |
ChemSpider | |
ECHA InfoCard | 100.155.728 |
KEGG | |
MeSH | Succinic+semialdehyde |
PubChem
CID
|
|
UNII | |
CompTox Dashboard (
EPA)
|
|
| |
| |
Properties | |
C4H6O3 | |
Molar mass | 102.089 g/mol |
Appearance | oil |
Boiling point | 135 °C (275 °F; 408 K) at 14 mmHg |
soluble in water, ethanol, benzene, diethyl ether [1] | |
Except where otherwise noted, data are given for materials in their
standard state (at 25 °C [77 °F], 100 kPa).
|
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]