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Literature summary extracted from

  • Kim, K.J.; Pearl, P.; Jensen, K.; Snead, O.C.; Malaspina, P.; Jakobs, C.; Gibson, K.M.
    Succinic semialdehyde dehydrogenase (SSADH): biochemical-molecular-clinical disease mechanisms, redox regulation and functional significance (2011), Antioxid. Redox Signal., 15, 691-718.
    View publication on PubMedView publication on EuropePMC

Crystallization (Commentary)

EC Number Crystallization (Comment) Organism
1.2.1.24
-
 Mus musculus
1.2.1.24
-
 Homo sapiens

Protein Variants

EC Number Protein Variants Comment Organism
1.2.1.24 A273S mutant with activity reduction Homo sapiens
1.2.1.24 C223R the mutation is associated with gamma-hydroxybutyric aciduria Homo sapiens
1.2.1.24 C340A inactive mutant that cannot form a disulfide bond even under strong reducing conditions Homo sapiens
1.2.1.24 C342A catalytically functional mutant that cannot form a disulfide bond even under strong reducing conditions Homo sapiens
1.2.1.24 G36R mutant with activity reduction Homo sapiens
1.2.1.24 H180Y mutant with activity reduction Homo sapiens
1.2.1.24 P182L mutant with significant activity reduction Homo sapiens

KM Value [mM]

EC Number KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
1.2.1.24 0.001
-
 succinate semialdehyde pH and temperature not specified in the publication Homo sapiens
1.2.1.24 0.003
-
 succinate semialdehyde pH and temperature not specified in the publication Mus musculus
1.2.1.24 0.04
-
 NAD+ pH and temperature not specified in the publication Homo sapiens
1.2.1.24 0.227
-
 NAD+ pH and temperature not specified in the publication Mus musculus

Localization

EC Number Localization Comment Organism GeneOntology No. Textmining
1.2.1.24 mitochondrial matrix
-
 Mus musculus 5759
-
1.2.1.24 mitochondrial matrix
-
 Homo sapiens 5759
-

Molecular Weight [Da]

EC Number Molecular Weight [Da] Molecular Weight Maximum [Da] Comment Organism
1.2.1.24 58000
-
 4 * 58000 Homo sapiens
1.2.1.24 191000
-
-
 Homo sapiens

Natural Substrates/ Products (Substrates)

EC Number Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
1.2.1.24 4-hydroxy-trans-2-nonenal + NAD+ + H2O Mus musculus
-
 4-hydroxy-trans-2-nonenoate + NADH + H+
-
 ?
1.2.1.24 4-hydroxy-trans-2-nonenal + NAD+ + H2O Homo sapiens
-
 4-hydroxy-trans-2-nonenoate + NADH + H+
-
 ?
1.2.1.24 succinate semialdehyde + NAD+ + H2O Mus musculus
-
 succinate + NADH + H+
-
 ?
1.2.1.24 succinate semialdehyde + NAD+ + H2O Homo sapiens
-
 succinate + NADH + H+
-
 ?

Organism

EC Number Organism UniProt Comment Textmining
1.2.1.24 Homo sapiens P51649
-
-
1.2.1.24 Mus musculus
-
-
-

Oxidation Stability

EC Number Oxidation Stability Organism
1.2.1.24 when reduced wild type SSADH is treated with hydrogen peroxide, the protein is almost completely inactivated and recovers its activity when the environment is switched back to a reduced state Mus musculus
1.2.1.24 when reduced wild type SSADH is treated with hydrogen peroxide, the protein is almost completely inactivated and recovers its activity when the environment was switched back to a reduced state Homo sapiens

Source Tissue

EC Number Source Tissue Comment Organism Textmining
1.2.1.24 blood
-
 Homo sapiens
-
1.2.1.24 brain SSADH is widely expressed throughout most brain regions, although a particularly strong expression is observed in the primary and secondary motor cortex, the amygdala, and the basal ganglia Mus musculus
-
1.2.1.24 brain SSADH is widely expressed throughout most brain regions, although a particularly strong expression is observed in the primary and secondary motor cortex, the amygdala, and the basal ganglia Homo sapiens
-
1.2.1.24 cerebellar Purkinje cell
-
 Homo sapiens
-
1.2.1.24 kidney fetal kidney Homo sapiens
-
1.2.1.24 liver
-
 Mus musculus
-
1.2.1.24 liver
-
 Homo sapiens
-
1.2.1.24 lymphoblast
-
 Homo sapiens
-

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
1.2.1.24 4-hydroxy-trans-2-nonenal + NAD+ + H2O
-
 Mus musculus 4-hydroxy-trans-2-nonenoate + NADH + H+
-
 ?
1.2.1.24 4-hydroxy-trans-2-nonenal + NAD+ + H2O
-
 Homo sapiens 4-hydroxy-trans-2-nonenoate + NADH + H+
-
 ?
1.2.1.24 succinate semialdehyde + NAD+ + H2O
-
 Mus musculus succinate + NADH + H+
-
 ?
1.2.1.24 succinate semialdehyde + NAD+ + H2O
-
 Homo sapiens succinate + NADH + H+
-
 ?

Subunits

EC Number Subunits Comment Organism
1.2.1.24 homotetramer
-
 Mus musculus
1.2.1.24 homotetramer 4 * 58000 Homo sapiens

Synonyms

EC Number Synonyms Comment Organism
1.2.1.24 aldehyde dehydrogenase 5a1
-
 Mus musculus
1.2.1.24 aldehyde dehydrogenase 5a1
-
 Homo sapiens
1.2.1.24 ALDH5A1
-
 Mus musculus
1.2.1.24 ALDH5A1
-
 Homo sapiens
1.2.1.24 SSADH
-
 Mus musculus
1.2.1.24 SSADH
-
 Homo sapiens
1.2.1.24 succinic semialdehyde dehydrogenase
-
 Mus musculus
1.2.1.24 succinic semialdehyde dehydrogenase
-
 Homo sapiens

Cofactor

EC Number Cofactor Comment Organism Structure
1.2.1.24 NAD+
-
 Mus musculus
1.2.1.24 NAD+
-
 Homo sapiens

General Information

EC Number General Information Comment Organism
1.2.1.24 malfunction at approximately postnatal day 16-22 SSADH-deficient mice display ataxia and loss of motor control, and develop generalized seizures leading to rapid death by the fourth week of life. D-2-hydroxyglutarate and 4,5-dihydroxyhexanoic acid are elevated in SSADH-deficient mice. SSADH-deficient mice demonstrate a 20% reduction in the ethanolamine glycerophospholipid content as compared to wild type littermates while other brain phospholipids (choline glycerophospholipid, phosphatidylserine and phosphatidylinositol) are not affected Mus musculus
1.2.1.24 malfunction SSADH enzyme activity is deficient in patients with gamma-hydroxybutyric aciduria Homo sapiens
1.2.1.24 metabolism under normal physiological conditions, SSADH works in tandem with GABA transaminase to convert the carbon backbone of gamma-aminobutyric acid to succinate, the latter a source of energy within the tricarboxylic acid cycle. SSADH, in brain, is the major aldehyde dehydrogenase responsible for 4-hydroxy-trans-2-nonenal disposition, but only a minor contributor to its metabolism in liver Mus musculus
1.2.1.24 metabolism under normal physiological conditions, SSADH works in tandem with GABA transaminase to convert the carbon backbone of gamma-aminobutyric acid to succinate, the latter a source of energy within the tricarboxylic acid cycle. SSADH, in brain, is the major aldehyde dehydrogenase responsible for 4-hydroxy-trans-2-nonenal disposition, but only a minor contributor to its metabolism in liver Homo sapiens