Compound: Succinic semialdehyde
SMILES: O=C(O)CCC=O
InChIKey: UIUJIQZEACWQSV-UHFFFAOYSA-N
Synonyms: Succinic semialdehyde, 4-Oxobutanoic acid, 692-29-5, Succinic acid semialdehyde, beta-Formylpropionic acid, gamma-Oxybutyric acid, 3-Formylpropanoic acid, 3-Formylpropionic acid, 4-oxo-butanoic acid, succinate semialdehyde, M73BX3CPMU, DTXSID00219231, CHEBI:16265, RefChem:186337, DTXCID20141722, 627-360-7, Succinaldehydic acid, Butryaldehydic acid, 4-Oxobutyric acid, Butanoic acid, 4-oxo-, 4-oxo-butyric acid, Semi-aldehyde succinique [French], BRN 1745187, Succinic semialdehyde monomer, b-Formylpropionic Acid, semialdehyde succinique, 4-03-00-01554 (Beilstein Handbook Reference), Semi-aldehyde succinique, UNII-M73BX3CPMU, Butryaldehydate, C00232, Succinaldehydate, gamma-Oxybutyrate, 4-Oxobutanoicacid, UIUJIQZEACWQSV-UHFFFAOYSA-N, 3-Formylpropanoate, Succinicsemialdehyde, 4-ketobutyric acid, beta-Formylpropionate, SCHEMBL42879, SCHEMBL59553, SCHEMBL536763, SCHEMBL3172501, CHEMBL1615238, SCHEMBL10354011, SCHEMBL27521926, LMFA06000118, MFCD00010579, AKOS006223730, FS151074, PD026754, DB-055240, HY-104071, CS-0028111, NS00015188, EN300-66985, Q4602542, 75002101-E68F-401B-8773-972512002B60
Theoretical properties:
molecular_formula: C4H6O3
molecular_weight: 102.09 g/mol
exact_mass: 102.031694049 g/mol
formal_charge: 0
hydrogen_bond_donor_count: 1
hydrogen_bond_acceptor_count: 3
rotatable_bond_count: 3
heavy_atom_count: 7
topological_polar_surface_area: 54.4 Å²
isotope_atom_count: 0
covalently_bonded_unit_count: 1
Physical properties:
Descriptions:
pubchem: Succinic semialdehyde is an aldehydic acid. It has a role as a mouse metabolite and an Escherichia coli metabolite. It is a conjugate acid of a 4-oxobutanoate.
chembl: Unnamed Compound is a small molecule listed in the ChEMBL database but has not progressed to an approved clinical phase.
wikipedia: Succinic semialdehyde dehydrogenase deficiency (SSADHD) is a rare autosomal recessive disorder of the degradation pathway of the inhibitory neurotransmitter γ-aminobutyric acid, or GABA.  The disorder has been identified in approximately 350 families, with a significant proportion being consanguineous families.  The first case was identified in 1981 and published in a Dutch clinical chemistry journal that highlighted a number of neurological conditions such as delayed intellectual, motor, speech, and language as the most common manifestations. Later cases reported in the early 1990s began to show that hypotonia, hyporeflexia, seizures, and a nonprogressive ataxia were frequent clinical features as well.
SSADH deficiency is caused by an enzyme deficiency in GABA degradation. Under normal conditions, SSADH works with the enzyme GABA transaminase to convert GABA to succinic acid. Succinic acid can then be utilized for energy production via the Krebs cycle. However, because of the deficiency, the final intermediate of the GABA degradation pathway, succinic semialdehyde, accumulates and cannot be oxidized to succinic acid and is therefore reduced to gamma-hydroxybutyric acid (GHB) by gamma-hydroxybutyric dehydrogenase. This causes elevations in GHB and is believed to be the trademark of this disorder and cause for the neurological manifestations seen.