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Information on EC 1.2.1.24 - succinate-semialdehyde dehydrogenase (NAD+) and Organism(s) Mus musculus and UniProt Accession Q8BWF0

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EC Tree
IUBMB Comments
This enzyme participates in the degradation of glutamate and 4-aminobutyrate. It is similar to EC 1.2.1.79 [succinate-semialdehyde dehydrogenase (NADP+)], and EC 1.2.1.16 [succinate-semialdehyde dehydrogenase (NAD(P)+)], but is specific for NAD+.
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This record set is specific for:
Mus musculus
UNIPROT: Q8BWF0
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Word Map
The taxonomic range for the selected organisms is: Mus musculus
The expected taxonomic range for this enzyme is: Eukaryota, Bacteria, Archaea
Synonyms
aldh5a1, aldh5a, aldehyde dehydrogenase 5a1, nad(+)-dependent succinic semialdehyde dehydrogenase, alphakgsa dehydrogenase, ssaldh, sso1629, more
SYNONYM
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
succinic semialdehyde dehydrogenase
-
aldehyde dehydrogenase 5a1
-
-
ALDH5A1
-
-
dehydrogenase, succinate semialdehyde
-
-
-
-
NAD(+)-dependent succinic semialdehyde dehydrogenase
-
-
-
-
SSADH
succinate semialdehyde dehydrogenase
succinate semialdehyde:NAD+ oxidoreductase
-
-
-
-
succinic semialdehyde dehydrogenase
succinyl semialdehyde dehydrogenase
-
-
-
-
REACTION TYPE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
redox reaction
-
-
-
-
oxidation
-
-
-
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reduction
-
-
-
-
SYSTEMATIC NAME
IUBMB Comments
succinate-semialdehyde:NAD+ oxidoreductase
This enzyme participates in the degradation of glutamate and 4-aminobutyrate. It is similar to EC 1.2.1.79 [succinate-semialdehyde dehydrogenase (NADP+)], and EC 1.2.1.16 [succinate-semialdehyde dehydrogenase (NAD(P)+)], but is specific for NAD+.
CAS REGISTRY NUMBER
COMMENTARY hide
9028-95-9
-
SUBSTRATE
PRODUCT                       
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate) hide
LITERATURE
(Substrate)
COMMENTARY
(Product) hide
LITERATURE
(Product)
Reversibility
r=reversible
ir=irreversible
?=not specified
2-carboxybenzaldehyde + NAD+ + H2O
2-carboxybenzoate + NADH + H+
show the reaction diagram
about 1% activity compared to succinate semialdehyde
-
-
?
2-hydroxybenzaldehyde + NAD+ + H2O
2-hydroxybenzoate + NADH + H+
show the reaction diagram
about 1% activity compared to succinate semialdehyde
-
-
?
2-nitrobenzaldehyde + NAD+ + H2O
2-nitrobenzoate + NADH + H+
show the reaction diagram
about 3% activity compared to succinate semialdehyde
-
-
?
2-phenylacetaldehyde + NAD+ + H2O
2-phenylbenzoate + NADH + H+
show the reaction diagram
about 5% activity compared to succinate semialdehyde
-
-
?
2-tolualdehyde + NAD+ + H2O
2-carboxytoluene + NADH + H+
show the reaction diagram
about 1% activity compared to succinate semialdehyde
-
-
?
3-carboxybenzaldehyde + NAD+ + H2O
3-carboxybenzoate + NADH + H+
show the reaction diagram
about 20% activity compared to succinate semialdehyde
-
-
?
3-hydroxybenzaldehyde + NAD+ + H2O
3-hydroxybenzoate + NADH + H+
show the reaction diagram
3-methoxy-benzaldehyde + NAD+ + H2O
3-methoxy-benzoate + NADH + H+
show the reaction diagram
about 5% activity compared to succinate semialdehyde
-
-
?
3-methylbenzaldehyde + NAD+ + H2O
3-methylbenzoate + NADH + H+
show the reaction diagram
about 3% activity compared to succinate semialdehyde
-
-
?
3-nitrobenzaldehyde + NAD+ + H2O
3-nitrobenzoate + NADH + H+
show the reaction diagram
about 20% activity compared to succinate semialdehyde
-
-
?
4-carboxybenzaldehyde + NAD+ + H2O
4-carboxybenzoate + NADH + H+
show the reaction diagram
about 10% activity compared to succinate semialdehyde
-
-
?
4-methoxy-benzaldehyde + NAD+ + H2O
4-methoxy-benzoate + NADH + H+
show the reaction diagram
about 1% activity compared to succinate semialdehyde
-
-
?
4-nitrobenzaldehyde + NAD+ + H2O
4-nitrobenzoate + NADH + H+
show the reaction diagram
about 15% activity compared to succinate semialdehyde
-
-
?
acetaldehyde + NAD+ + H2O
acetate + NADH + H+
show the reaction diagram
about 5% activity compared to succinate semialdehyde
-
-
?
benzaldehyde + NAD+ + H2O
benzoate + NADH + H+
show the reaction diagram
about 5% activity compared to succinate semialdehyde
-
-
?
glyoxylic acid + NAD+ + H2O
2-phenylbenzoate + NADH + H+
show the reaction diagram
about 1% activity compared to succinate semialdehyde
-
-
?
iso-butanal + NAD+ + H2O
? + NADH + H+
show the reaction diagram
less than 10% activity compared to succinate semialdehyde
-
-
?
iso-pentanal + NAD+ + H2O
? + NADH + H+
show the reaction diagram
about 2% activity compared to succinate semialdehyde
-
-
?
methyl 3-formylbenzoate + NAD+ + H2O
3-(methoxycarbonyl)benzoate + NADH + H+
show the reaction diagram
about 20% activity compared to succinate semialdehyde
-
-
?
methyl 4-formylbenzoate + NAD+ + H2O
4-(methoxycarbonyl)benzoate + NADH + H+
show the reaction diagram
about 15% activity compared to succinate semialdehyde
-
-
?
n-butanal + NAD+ + H2O
butanoate + NADH + H+
show the reaction diagram
about 10% activity compared to succinate semialdehyde
-
-
?
n-hexanal + NAD+ + H2O
hexanoate + NADH + H+
show the reaction diagram
less than 10% activity compared to succinate semialdehyde
-
-
?
n-octanal + NAD+ + H2O
n-octanoate + NADH + H+
show the reaction diagram
less than 10% activity compared to succinate semialdehyde
-
-
?
n-pentanal + NAD+ + H2O
n-pentanoate + NADH + H+
show the reaction diagram
less than 20% activity compared to succinate semialdehyde
-
-
?
propanal + NAD+ + H2O
propanoate + NADH + H+
show the reaction diagram
less than 10% activity compared to succinate semialdehyde
-
-
?
succinate semialdehyde + NAD+ + H2O
succinate + NADH + 2 H+
show the reaction diagram
-
-
-
?
succinate semialdehyde + NAD+ + H2O
succinate + NADH + H+
show the reaction diagram
succinate semialdehyde + NADP+ + H2O
succinate + NADPH + H+
show the reaction diagram
NAD+ is the more efficient coenzyme compared to NADP+, but the preference is not exclusive
-
-
?
4-hydroxy-trans-2-nonenal + NAD+ + H2O
4-hydroxy-trans-2-nonenoate + NADH + H+
show the reaction diagram
-
-
-
-
?
succinate semialdehyde + NAD+ + H2O
succinate + NADH + H+
show the reaction diagram
additional information
?
-
no activity towards 2-methoxybenzaldehyde, and 4-tolualdehyde
-
-
?
NATURAL SUBSTRATE
NATURAL PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate) hide
LITERATURE
(Substrate)
COMMENTARY
(Product) hide
LITERATURE
(Product)
REVERSIBILITY
r=reversible
ir=irreversible
?=not specified
succinate semialdehyde + NAD+ + H2O
succinate + NADH + 2 H+
show the reaction diagram
-
-
-
?
4-hydroxy-trans-2-nonenal + NAD+ + H2O
4-hydroxy-trans-2-nonenoate + NADH + H+
show the reaction diagram
-
-
-
-
?
succinate semialdehyde + NAD+ + H2O
succinate + NADH + H+
show the reaction diagram
COFACTOR
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
INHIBITOR
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
4-hydroxybenzaldehyde
more than 95% inhibition at 0.05 mM
4-methoxybenzaldehyde
-
4-tolualdehyde
-
succinate semialdehyde
the enzyme loses more than 80% of its enzymatic potential at 0.5 mM and shows 8% residual activity at 3 mM succinate semialdehyde
KM VALUE [mM]
SUBSTRATE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
0.0331
3-carboxybenzaldehyde
in 100 mM Na2HPO4/NaH2PO4 buffer, 1 mM dithiothreitol, at pH 8.5 and 22°C
0.287
3-Nitrobenzaldehyde
in 100 mM Na2HPO4/NaH2PO4 buffer, 1 mM dithiothreitol, at pH 8.5 and 22°C
0.0151 - 0.115
4-carboxybenzaldehyde
0.156
4-nitrobenzaldehyde
in 100 mM Na2HPO4/NaH2PO4 buffer, 1 mM dithiothreitol, at pH 8.5 and 22°C
0.218
methyl 3-formylbenzoate
in 100 mM Na2HPO4/NaH2PO4 buffer, 1 mM dithiothreitol, at pH 8.5 and 22°C
0.061
n-Butanal
in 100 mM Na2HPO4/NaH2PO4 buffer, 1 mM dithiothreitol, at pH 8.5 and 22°C
0.0253
n-hexanal
in 100 mM Na2HPO4/NaH2PO4 buffer, 1 mM dithiothreitol, at pH 8.5 and 22°C
0.137
n-octanal
in 100 mM Na2HPO4/NaH2PO4 buffer, 1 mM dithiothreitol, at pH 8.5 and 22°C
0.0287
n-Pentanal
in 100 mM Na2HPO4/NaH2PO4 buffer, 1 mM dithiothreitol, at pH 8.5 and 22°C
0.227
NAD+
in 100 mM Na2HPO4/NaH2PO4 buffer, 1 mM dithiothreitol, at pH 8.5 and 22°C
0.616
NADP+
in 100 mM Na2HPO4/NaH2PO4 buffer, 1 mM dithiothreitol, at pH 8.5 and 22°C
1.212
propanal
in 100 mM Na2HPO4/NaH2PO4 buffer, 1 mM dithiothreitol, at pH 8.5 and 22°C
0.00247
succinate semialdehyde
in 100 mM Na2HPO4/NaH2PO4 buffer, 1 mM dithiothreitol, at pH 8.5 and 22°C
0.227
NAD+
-
pH and temperature not specified in the publication
0.003
succinate semialdehyde
-
pH and temperature not specified in the publication
IC50 VALUE [mM]
INHIBITOR
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
2
4-methoxybenzaldehyde
Mus musculus
-
1
4-tolualdehyde
Mus musculus
-
ORGANISM
COMMENTARY hide
LITERATURE
UNIPROT
SEQUENCE DB
SOURCE
-
Uniprot
Manually annotated by BRENDA team
SOURCE TISSUE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
SOURCE
-
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
Manually annotated by BRENDA team
LOCALIZATION
ORGANISM
UNIPROT
COMMENTARY hide
GeneOntology No.
LITERATURE
SOURCE
GENERAL INFORMATION
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
malfunction
succinic semialdehyde dehydrogenase (SSADH) deficiency (SSADHD) is a heritable disorder of gamma-aminobutyric acid (GABA) metabolism associating with elevated GABA and gamma-hydroxybutyrate (GHB). The heterogeneous phenotype ranges from mild to severe, including developmental delay, movement abnormalities, neuropsychiatric morbidity, and seizures murine succinic semialdehyde dehydrogenase deficiency (SSADHD) manifests with high concentrations of gamma-aminobutyric acid (GABA) and gamma-hydroxybutyrate (GHB) and low glutamine in the brain
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
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
UNIPROT
ENTRY NAME
ORGANISM
NO. OF AA
NO. OF TRANSM. HELICES
MOLECULAR WEIGHT[Da]
SOURCE
SEQUENCE
LOCALIZATION PREDICTION?
SSDH_MOUSE
523
0
55968
Swiss-Prot
Mitochondrion (Reliability: 2)
MOLECULAR WEIGHT
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
98000
SDS-PAGE
SUBUNIT
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
homotetramer
-
-
CRYSTALLIZATION (Commentary)
ORGANISM
UNIPROT
LITERATURE
PROTEIN VARIANTS
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
additional information
OXIDATION STABILITY
ORGANISM
UNIPROT
LITERATURE
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
-
710898
PURIFICATION (Commentary)
ORGANISM
UNIPROT
LITERATURE
Ni2+-NTA agarose column chromatography
CLONED (Commentary)
ORGANISM
UNIPROT
LITERATURE
expressed in Escherichia coli M15 cells
APPLICATION
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
medicine
SSADH (Aldh5a1) deficiency is a rare autosomal recessive disease. Hippocampal and cortical dysfunction in Aldh5a1-/- brain, but no evidence that accumulating key metabolites of SSADH deficiency directly induce impairment of energy metabolism
REF.
AUTHORS
TITLE
JOURNAL
VOL.
PAGES
YEAR
ORGANISM (UNIPROT)
PUBMED ID
SOURCE
Sauer, S.W.; Koelker, S.; Hoffmann, G.F.; Ten Brink, H.J.; Jakobs, C.; Gibson, K.M.; Okun, J.G.
Enzymatic and metabolic evidence for a region specific mitochondrial dysfunction in brains of murine succinic semialdehyde dehydrogenase deficiency (Aldh5a1-/- mice)
Neurochem. Int.
50
653-659
2007
Mus musculus (Q8BWF0)
Manually annotated by BRENDA team
Jansen, E.E.; Struys, E.; Jakobs, C.; Hager, E.; Snead, O.C.; Gibson, K.M.
Neurotransmitter alterations in embryonic succinate semialdehyde dehydrogenase (SSADH) deficiency suggest a heightened excitatory state during development
BMC Dev. Biol.
8
112
2008
Mus musculus
Manually annotated by BRENDA team
Stewart, L.S.; Nylen, K.J.; Persinger, M.A.; Cortez, M.A.; Gibson, K.M.; Snead, O.C.
Circadian distribution of generalized tonic-clonic seizures associated with murine succinic semialdehyde dehydrogenase deficiency, a disorder of GABA metabolism
Epilepsy Behav.
13
290-294
2008
Mus musculus
Manually annotated by BRENDA team
Nylen, K.; Velazquez, J.L.; Likhodii, S.S.; Cortez, M.A.; Shen, L.; Leshchenko, Y.; Adeli, K.; Gibson, K.M.; Burnham, W.M.; Snead, O.C.
A ketogenic diet rescues the murine succinic semialdehyde dehydrogenase deficient phenotype
Exp. Neurol.
210
449-457
2008
Mus musculus
Manually annotated by BRENDA team
Pearl, P.L.; Gibson, K.M.; Cortez, M.A.; Wu, Y.; Carter Snead, O.; Knerr, I.; Forester, K.; Pettiford, J.M.; Jakobs, C.; Theodore, W.H.
Succinic semialdehyde dehydrogenase deficiency: lessons from mice and men
J. Inherit. Metab. Dis.
32
343-352
2009
Homo sapiens, Mus musculus
Manually annotated by BRENDA team
Rothacker, B.; Werr, M.; Ilg, T.
Succinic semialdehyde dehydrogenase from the parasitic cattle tick Rhipicephalus microplus: gene identification, biochemical characterization and comparison with the mouse ortholog
Mol. Biochem. Parasitol.
161
32-43
2008
Mus musculus (Q8BWF0), Rhipicephalus microplus
Manually annotated by BRENDA team
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
Antioxid. Redox Signal.
15
691-718
2011
Homo sapiens (P51649), Homo sapiens, Mus musculus
Manually annotated by BRENDA team
Brown, M.N.; Walters, D.C.; Schmidt, M.A.; Hill, J.; McConnell, A.; Jansen, E.E.W.; Salomons, G.S.; Arning, E.; Bottiglieri, T.; Gibson, K.M.; Roullet, J.B.
Maternal glutamine supplementation in murine succinic semialdehyde dehydrogenase deficiency, a disorder of gamma-aminobutyric acid metabolism
J. Inherit. Metab. Dis.
42
1030-1039
2019
Mus musculus (Q8BWF0), Mus musculus C57BL/6 (Q8BWF0)
Manually annotated by BRENDA team