Information on EC 1.1.99.24 - hydroxyacid-oxoacid transhydrogenase

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The enzyme appears in viruses and cellular organisms

EC NUMBER
COMMENTARY hide
1.1.99.24
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RECOMMENDED NAME
GeneOntology No.
hydroxyacid-oxoacid transhydrogenase
REACTION
REACTION DIAGRAM
COMMENTARY hide
ORGANISM
UNIPROT
LITERATURE
(S)-3-hydroxybutanoate + 2-oxoglutarate = acetoacetate + (R)-2-hydroxyglutarate
show the reaction diagram
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REACTION TYPE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
oxidation
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redox reaction
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reduction
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transhydrogenation
SYSTEMATIC NAME
IUBMB Comments
(S)-3-hydroxybutanoate:2-oxoglutarate oxidoreductase
4-Hydroxybutanoate and (R)-2-hydroxyglutarate can also act as donors; 4-oxobutanoate can also act as acceptor.
CAS REGISTRY NUMBER
COMMENTARY hide
117698-31-4
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ORGANISM
COMMENTARY hide
LITERATURE
UNIPROT
SEQUENCE DB
SOURCE
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Manually annotated by BRENDA team
Sprague-Dawley strain
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-
Manually annotated by BRENDA team
SUBSTRATE
PRODUCT                       
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate) hide
LITERATURE
(Substrate)
COMMENTARY
(Product) hide
LITERATURE
(Product)
Reversibility
r=reversible
ir=irreversible
?=not specified
3-hydroxybutyrate + 2-oxoglutarate
succinic semialdehyde + D-2-hydroxyglutarate
show the reaction diagram
4-hydroxybutanoic acid + 2-oxoglutarate
succinic semialdehyde + D-2-hydroxyglutarate
show the reaction diagram
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-
-
?
4-hydroxybutyrate + 2-oxoglutarate
D-2-hydroxyglutarate + succinate semialdehyde
show the reaction diagram
4-hydroxybutyrate + 2-oxoglutarate
succinic semialdehyde + 2-hydroxyglutarate
show the reaction diagram
4-hydroxybutyrate + 2-oxoglutarate
succinic semialdehyde + D-2-hydroxyglutarate
show the reaction diagram
D-2-hydroxyglutarate + succinic semialdehyde
2-oxoglutarate + 4-hydroxybutyrate
show the reaction diagram
L-3-hydroxybutyrate + 2-oxoglutarate
acetoacetate + 2-oxoglutarate
show the reaction diagram
L-3-hydroxybutyrate + succinic semialdehyde
acetoacetate + 4-hydroxybutyrate
show the reaction diagram
additional information
?
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D-2-hydroxyglutaric aciduria is not associated to the enzyme activity
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NATURAL SUBSTRATES
NATURAL PRODUCTS
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate) hide
LITERATURE
(Substrate)
COMMENTARY
(Product) hide
LITERATURE
(Product)
REVERSIBILITY
r=reversible
ir=irreversible
?=not specified
3-hydroxybutyrate + 2-oxoglutarate
succinic semialdehyde + D-2-hydroxyglutarate
show the reaction diagram
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-
-
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r
4-hydroxybutyrate + 2-oxoglutarate
D-2-hydroxyglutarate + succinate semialdehyde
show the reaction diagram
4-hydroxybutyrate + 2-oxoglutarate
succinic semialdehyde + D-2-hydroxyglutarate
show the reaction diagram
additional information
?
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D-2-hydroxyglutaric aciduria is not associated to the enzyme activity
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COFACTOR
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
NAD(P)+
2-oxoglutarate serves as an intermediate acceptor to generate NAD(P)H
additional information
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METALS and IONS
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
additional information
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bi or trivalent, no stimulation with 0.01-0.05 mM Fe2+, Fe3+, Co2+, Ni2+, Mn2+, Zn2+, Mg2+ or Ca2+; not affected by 0.5 mM Fe2+, Fe3+, Co2+, Ni2+, Mn2+, and Zn2+, or 1 mM Mg2+ and Ca2+
INHIBITORS
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
1,10-phenanthroline
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; IC50: 0.75 mM
cyanide
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; IC50: 0.075 mM
additional information
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ACTIVATING COMPOUND
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
additional information
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no effect by EDTA and EGTA
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KM VALUE [mM]
SUBSTRATE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
0.018 - 0.085
2-oxoglutarate
0.06 - 0.3
4-hydroxybutyrate
4.5
D-2-hydroxybutyrate
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no activity with L-isomer, 30°C, pH 7.1
0.42 - 4.5
D-2-hydroxyglutarate
3
L-3-hydroxybutyrate
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0.0046 - 0.01
Succinic semialdehyde
additional information
additional information
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no activity with L-isomer of 2-hydroxybutyrate, Km for succinate semialdehyde is below 0.01 mM, 30°C, pH 7.1
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Ki VALUE [mM]
INHIBITOR
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
0.75
1,10-phenanthroline
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30°C, pH 7.1
0.075
cyanide
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30°C, pH 7.1
IC50 VALUE [mM]
INHIBITOR
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
0.75
1,10-phenanthroline
Rattus norvegicus
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IC50: 0.75 mM
0.075
cyanide
Rattus norvegicus
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IC50: 0.075 mM
SPECIFIC ACTIVITY [µmol/min/mg]
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
0.0013
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recombinant soluble enzyme in HEK-293 cells
additional information
pH OPTIMUM
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
7.5
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assay at
TEMPERATURE OPTIMUM
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
SOURCE TISSUE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
SOURCE
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of healthy subjects and patients with D-2-hydroxyglutaric aciduria
Manually annotated by BRENDA team
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highest activity in liver and kidney and an intermediate activity in heart; low enzyme activity
Manually annotated by BRENDA team
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mouse homologue expressed in
Manually annotated by BRENDA team
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very low enzyme activity
Manually annotated by BRENDA team
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low enzyme activity
Manually annotated by BRENDA team
additional information
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no activity in brain and skeletal muscle; tissue distribution, overview
Manually annotated by BRENDA team
LOCALIZATION
ORGANISM
UNIPROT
COMMENTARY hide
GeneOntology No.
LITERATURE
SOURCE
MOLECULAR WEIGHT
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
45000 - 50000
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45000 - 55000
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gel filtration
50000
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1 * 50000, about, SDS-PAGE
SUBUNITS
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
monomer
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1 * 50000, about, SDS-PAGE
GENERAL STABILITY
ORGANISM
UNIPROT
LITERATURE
NAD+ stabilizes during ammonium sulfate precipitation
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Purification/COMMENTARY
ORGANISM
UNIPROT
LITERATURE
1300fold, partial from liver; native enzyme from liver 1300fold by precipitation using PEG 6000, anion exchange and hydrophobic interaction chromatography, and gel filtration
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Cloned/COMMENTARY
ORGANISM
UNIPROT
LITERATURE
DNA and amino acid sequence determination and analysis, expression in HEK-293 cells as soluble protein
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