Crystallization (Comment) | Organism |
---|---|
- |
Mus musculus |
- |
Homo sapiens |
Protein Variants | Comment | Organism |
---|---|---|
A273S | mutant with activity reduction | Homo sapiens |
C223R | the mutation is associated with gamma-hydroxybutyric aciduria | Homo sapiens |
C340A | inactive mutant that cannot form a disulfide bond even under strong reducing conditions | Homo sapiens |
C342A | catalytically functional mutant that cannot form a disulfide bond even under strong reducing conditions | Homo sapiens |
G36R | mutant with activity reduction | Homo sapiens |
H180Y | mutant with activity reduction | Homo sapiens |
P182L | mutant with significant activity reduction | Homo sapiens |
KM Value [mM] | KM Value Maximum [mM] | Substrate | Comment | Organism | Structure |
---|---|---|---|---|---|
0.001 | - |
succinate semialdehyde | pH and temperature not specified in the publication | Homo sapiens | |
0.003 | - |
succinate semialdehyde | pH and temperature not specified in the publication | Mus musculus | |
0.04 | - |
NAD+ | pH and temperature not specified in the publication | Homo sapiens | |
0.227 | - |
NAD+ | pH and temperature not specified in the publication | Mus musculus |
Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|
mitochondrial matrix | - |
Mus musculus | 5759 | - |
mitochondrial matrix | - |
Homo sapiens | 5759 | - |
Molecular Weight [Da] | Molecular Weight Maximum [Da] | Comment | Organism |
---|---|---|---|
58000 | - |
4 * 58000 | Homo sapiens |
191000 | - |
- |
Homo sapiens |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
4-hydroxy-trans-2-nonenal + NAD+ + H2O | Mus musculus | - |
4-hydroxy-trans-2-nonenoate + NADH + H+ | - |
? | |
4-hydroxy-trans-2-nonenal + NAD+ + H2O | Homo sapiens | - |
4-hydroxy-trans-2-nonenoate + NADH + H+ | - |
? | |
succinate semialdehyde + NAD+ + H2O | Mus musculus | - |
succinate + NADH + H+ | - |
? | |
succinate semialdehyde + NAD+ + H2O | Homo sapiens | - |
succinate + NADH + H+ | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | P51649 | - |
- |
Mus musculus | - |
- |
- |
Oxidation Stability | Organism |
---|---|
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 |
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 | Comment | Organism | Textmining |
---|---|---|---|
blood | - |
Homo sapiens | - |
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 | - |
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 | - |
cerebellar Purkinje cell | - |
Homo sapiens | - |
kidney | fetal kidney | Homo sapiens | - |
liver | - |
Mus musculus | - |
liver | - |
Homo sapiens | - |
lymphoblast | - |
Homo sapiens | - |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
4-hydroxy-trans-2-nonenal + NAD+ + H2O | - |
Mus musculus | 4-hydroxy-trans-2-nonenoate + NADH + H+ | - |
? | |
4-hydroxy-trans-2-nonenal + NAD+ + H2O | - |
Homo sapiens | 4-hydroxy-trans-2-nonenoate + NADH + H+ | - |
? | |
succinate semialdehyde + NAD+ + H2O | - |
Mus musculus | succinate + NADH + H+ | - |
? | |
succinate semialdehyde + NAD+ + H2O | - |
Homo sapiens | succinate + NADH + H+ | - |
? |
Subunits | Comment | Organism |
---|---|---|
homotetramer | - |
Mus musculus |
homotetramer | 4 * 58000 | Homo sapiens |
Synonyms | Comment | Organism |
---|---|---|
aldehyde dehydrogenase 5a1 | - |
Mus musculus |
aldehyde dehydrogenase 5a1 | - |
Homo sapiens |
ALDH5A1 | - |
Mus musculus |
ALDH5A1 | - |
Homo sapiens |
SSADH | - |
Mus musculus |
SSADH | - |
Homo sapiens |
succinic semialdehyde dehydrogenase | - |
Mus musculus |
succinic semialdehyde dehydrogenase | - |
Homo sapiens |
Cofactor | Comment | Organism | Structure |
---|---|---|---|
NAD+ | - |
Mus musculus | |
NAD+ | - |
Homo sapiens |
General Information | Comment | Organism |
---|---|---|
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 |
malfunction | SSADH enzyme activity is deficient in patients with gamma-hydroxybutyric aciduria | Homo sapiens |
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 |
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 |