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Information on EC 1.1.99.24 - hydroxyacid-oxoacid transhydrogenase
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1.1.99.24 hydroxyacid-oxoacid transhydrogenaseIUBMB Comments
4-Hydroxybutanoate and (R)-2-hydroxyglutarate can also act as donors; 4-oxobutanoate can also act as acceptor.
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Word Map
- 1.1.99.24
- gamma-hydroxybutyrate
- semialdehyde
- 2-hydroxyglutarate
-
iron-containing
-
acidurias
-
oncometabolite
- 2-ketoglutarate
- diagnostics
The enzyme appears in viruses and cellular organisms
Reaction Schemes
Synonyms
adhfe1, hydroxyacid-oxoacid transhydrogenase, alcohol dehydrogenase iron containing 1, 
more
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SYNONYM 
ORGANISM
UNIPROT
COMMENTARY 
LITERATURE
HOT
transhydrogenase, hydroxy acid-oxo acid
transhydrogenase, hydroxy acid-oxo acid
-
-
-
-
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REACTION
REACTION DIAGRAM
COMMENTARY
ORGANISM
UNIPROT
LITERATURE
(S)-3-hydroxybutanoate + 2-oxoglutarate = acetoacetate + (R)-2-hydroxyglutarate
-
-
-
-
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REACTION TYPE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
oxidation
-
-
-
-
redox reaction
-
-
-
-
reduction
-
-
-
-
transhydrogenation
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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.
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CAS REGISTRY NUMBER
COMMENTARY
117698-31-4
-
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SUBSTRATE
PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate)
(Substrate)
LITERATURE
(Substrate)
(Substrate)
COMMENTARY
(Product)
(Product)
LITERATURE
(Product)
(Product)
Reversibility
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
4-hydroxybutanoic acid + 2-oxoglutarate
succinic semialdehyde + D-2-hydroxyglutarate
-
-
-
?
additional information
?
-
-
D-2-hydroxyglutaric aciduria is not associated to the enzyme activity
-
-
?
(S)-3-hydroxybutanoate + 2-oxoglutarate
acetoacetate + (R)-2-hydroxyglutarate
-
-
-
-
?
(S)-3-hydroxybutanoate + 2-oxoglutarate
acetoacetate + (R)-2-hydroxyglutarate
-
-
-
-
?
(S)-3-hydroxybutanoate + 2-oxoglutarate
acetoacetate + (R)-2-hydroxyglutarate
-
-
-
?
(S)-3-hydroxybutanoate + 2-oxoglutarate
acetoacetate + (R)-2-hydroxyglutarate
-
-
-
?
(S)-3-hydroxybutanoate + 2-oxoglutarate
acetoacetate + (R)-2-hydroxyglutarate
-
-
-
?
(S)-3-hydroxybutanoate + 2-oxoglutarate
acetoacetate + (R)-2-hydroxyglutarate
-
-
-
?
(S)-3-hydroxybutanoate + 2-oxoglutarate
acetoacetate + (R)-2-hydroxyglutarate
-
-
-
?
(S)-3-hydroxybutanoate + 2-oxoglutarate
acetoacetate + (R)-2-hydroxyglutarate
-
-
-
-
?
(S)-3-hydroxybutanoate + 2-oxoglutarate
acetoacetate + (R)-2-hydroxyglutarate
-
-
-
?
(S)-3-hydroxybutanoate + 2-oxoglutarate
acetoacetate + (R)-2-hydroxyglutarate
-
-
-
?
(S)-3-hydroxybutanoate + 2-oxoglutarate
acetoacetate + (R)-2-hydroxyglutarate
-
-
-
?
(S)-3-hydroxybutanoate + 2-oxoglutarate
acetoacetate + (R)-2-hydroxyglutarate
-
-
-
?
(S)-3-hydroxybutanoate + 2-oxoglutarate
acetoacetate + (R)-2-hydroxyglutarate
-
-
-
?
(S)-3-hydroxybutanoate + 2-oxoglutarate
acetoacetate + (R)-2-hydroxyglutarate
-
-
-
?
3-hydroxybutyrate + 2-oxoglutarate
succinic semialdehyde + D-2-hydroxyglutarate
-
-
-
-
r
3-hydroxybutyrate + 2-oxoglutarate
succinic semialdehyde + D-2-hydroxyglutarate
-
radiolabeled substrate 3-hydroxybutyrate, stereospecific reaction
radiolabeled product D-2-hydroxyglutarate
-
r
4-hydroxybutyrate + 2-oxoglutarate
D-2-hydroxyglutarate + succinate semialdehyde
-
-
-
-
?
4-hydroxybutyrate + 2-oxoglutarate
D-2-hydroxyglutarate + succinate semialdehyde
-
-
-
-
?
4-hydroxybutyrate + 2-oxoglutarate
succinic semialdehyde + 2-hydroxyglutarate
-
-
-
r
4-hydroxybutyrate + 2-oxoglutarate
succinic semialdehyde + 2-hydroxyglutarate
-
hydrogen attached to hydroxyl-bearing C of 4-hydroxybutyrate is transferred to ketone-bearing C of 2-oxoglutarate, oxoacid not NAD+ or NADP+ acts as hydrogen acceptor
-
r
4-hydroxybutyrate + 2-oxoglutarate
succinic semialdehyde + 2-hydroxyglutarate
-
hydrogen attached to hydroxyl-bearing C of 4-hydroxybutyrate is transferred to ketone-bearing C of 2-oxoglutarate, oxoacid not NAD+ or NADP+ acts as hydrogen acceptor
-
r
4-hydroxybutyrate + 2-oxoglutarate
succinic semialdehyde + D-2-hydroxyglutarate
-
-
-
-
?
4-hydroxybutyrate + 2-oxoglutarate
succinic semialdehyde + D-2-hydroxyglutarate
-
recombinant enzyme in HEK-293 cells
-
-
?
4-hydroxybutyrate + 2-oxoglutarate
succinic semialdehyde + D-2-hydroxyglutarate
-
-
-
-
r
4-hydroxybutyrate + 2-oxoglutarate
succinic semialdehyde + D-2-hydroxyglutarate
-
stereospecific reaction, no activity with L-2-hydroxyglutarate
-
-
r
D-2-hydroxyglutarate + succinic semialdehyde
2-oxoglutarate + 4-hydroxybutyrate
-
-
-
?
D-2-hydroxyglutarate + succinic semialdehyde
2-oxoglutarate + 4-hydroxybutyrate
-
-
-
?
D-2-hydroxyglutarate + succinic semialdehyde
2-oxoglutarate + 4-hydroxybutyrate
-
-
-
?
L-3-hydroxybutyrate + 2-oxoglutarate
acetoacetate + 2-oxoglutarate
-
-
-
?
L-3-hydroxybutyrate + 2-oxoglutarate
acetoacetate + 2-oxoglutarate
-
oxidized at 33% the rate of 4-hydroxybutyrate
-
?
L-3-hydroxybutyrate + 2-oxoglutarate
acetoacetate + 2-oxoglutarate
-
oxidized at 33% the rate of 4-hydroxybutyrate
-
?
L-3-hydroxybutyrate + succinic semialdehyde
acetoacetate + 4-hydroxybutyrate
-
-
-
?
L-3-hydroxybutyrate + succinic semialdehyde
acetoacetate + 4-hydroxybutyrate
-
oxo-acids that can act as hydrogen acceptors are 2-oxoadipate, 24% as effective as 2-oxoglutarate and oxaloacetate, 19% as effective as 2-oxoglutarate, D-3-hydroxybutyrate, DL-2-hydroxybutyrate, D/L-malate, D/L-lactate, acetoacetate, 3-oxobutyrate, 3-oxoadipate do not act as hydrogen acceptors, poor acceptors are pyruvate and 2-oxobutyrate
-
?
L-3-hydroxybutyrate + succinic semialdehyde
acetoacetate + 4-hydroxybutyrate
-
62.5% of activity with D-2-hydroxyglutarate
-
?
L-3-hydroxybutyrate + succinic semialdehyde
acetoacetate + 4-hydroxybutyrate
-
oxo-acids that can act as hydrogen acceptors are 2-oxoadipate, 24% as effective as 2-oxoglutarate and oxaloacetate, 19% as effective as 2-oxoglutarate, D-3-hydroxybutyrate, DL-2-hydroxybutyrate, D/L-malate, D/L-lactate, acetoacetate, 3-oxobutyrate, 3-oxoadipate do not act as hydrogen acceptors, poor acceptors are pyruvate and 2-oxobutyrate
-
?
L-3-hydroxybutyrate + succinic semialdehyde
acetoacetate + 4-hydroxybutyrate
-
62.5% of activity with D-2-hydroxyglutarate
-
?
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NATURAL SUBSTRATE
NATURAL PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate)
(Substrate)
LITERATURE
(Substrate)
(Substrate)
COMMENTARY
(Product)
(Product)
LITERATURE
(Product)
(Product)
REVERSIBILITY
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
3-hydroxybutyrate + 2-oxoglutarate
succinic semialdehyde + D-2-hydroxyglutarate
-
-
-
-
r
additional information
?
-
-
D-2-hydroxyglutaric aciduria is not associated to the enzyme activity
-
-
?
(S)-3-hydroxybutanoate + 2-oxoglutarate
acetoacetate + (R)-2-hydroxyglutarate
-
-
-
-
?
(S)-3-hydroxybutanoate + 2-oxoglutarate
acetoacetate + (R)-2-hydroxyglutarate
-
-
-
-
?
(S)-3-hydroxybutanoate + 2-oxoglutarate
acetoacetate + (R)-2-hydroxyglutarate
-
-
-
?
(S)-3-hydroxybutanoate + 2-oxoglutarate
acetoacetate + (R)-2-hydroxyglutarate
-
-
-
?
(S)-3-hydroxybutanoate + 2-oxoglutarate
acetoacetate + (R)-2-hydroxyglutarate
-
-
-
?
(S)-3-hydroxybutanoate + 2-oxoglutarate
acetoacetate + (R)-2-hydroxyglutarate
-
-
-
?
(S)-3-hydroxybutanoate + 2-oxoglutarate
acetoacetate + (R)-2-hydroxyglutarate
-
-
-
?
(S)-3-hydroxybutanoate + 2-oxoglutarate
acetoacetate + (R)-2-hydroxyglutarate
-
-
-
-
?
(S)-3-hydroxybutanoate + 2-oxoglutarate
acetoacetate + (R)-2-hydroxyglutarate
-
-
-
?
(S)-3-hydroxybutanoate + 2-oxoglutarate
acetoacetate + (R)-2-hydroxyglutarate
-
-
-
?
(S)-3-hydroxybutanoate + 2-oxoglutarate
acetoacetate + (R)-2-hydroxyglutarate
-
-
-
?
(S)-3-hydroxybutanoate + 2-oxoglutarate
acetoacetate + (R)-2-hydroxyglutarate
-
-
-
?
(S)-3-hydroxybutanoate + 2-oxoglutarate
acetoacetate + (R)-2-hydroxyglutarate
-
-
-
?
(S)-3-hydroxybutanoate + 2-oxoglutarate
acetoacetate + (R)-2-hydroxyglutarate
-
-
-
?
4-hydroxybutyrate + 2-oxoglutarate
D-2-hydroxyglutarate + succinate semialdehyde
-
-
-
-
?
4-hydroxybutyrate + 2-oxoglutarate
D-2-hydroxyglutarate + succinate semialdehyde
-
-
-
-
?
4-hydroxybutyrate + 2-oxoglutarate
succinic semialdehyde + D-2-hydroxyglutarate
-
recombinant enzyme in HEK-293 cells
-
-
?
4-hydroxybutyrate + 2-oxoglutarate
succinic semialdehyde + D-2-hydroxyglutarate
-
-
-
-
r
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COFACTOR
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
NAD(P)+
2-oxoglutarate serves as an intermediate acceptor to generate NAD(P)H
additional information
-
enzymatic activity is unaffected by NAD+ or NADP+ up to 1 mM
-
additional information
-
enzymatic activity is unaffected by NAD+ or NADP+ up to 1 mM
-
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METALS and IONS
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
additional information
additional information
-
bi or trivalent, no stimulation with 0.01-0.05 mM Fe2+, Fe3+, Co2+, Ni2+, Mn2+, Zn2+, Mg2+ or Ca2+
additional information
-
not affected by 0.5 mM Fe2+, Fe3+, Co2+, Ni2+, Mn2+, and Zn2+, or 1 mM Mg2+ and Ca2+
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INHIBITOR
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
additional information
-
no effect by EDTA and EGTA; no inhibition with EDTA, EGTA
-
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ACTIVATING COMPOUND
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
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DISEASE
TITLE OF PUBLICATION
LINK TO PUBMED
Adenoma
Alcohol dehydrogenase, iron containing, 1 promoter hypermethylation associated with colorectal cancer differentiation.
Adenoma
Genome-wide DNA methylation profiles of low- and high-grade adenoma reveals potential biomarkers for early detection of colorectal carcinoma.
Adenoma
Identification and validation of highly frequent CpG island hypermethylation in colorectal adenomas and carcinomas.
alcohol dehydrogenase deficiency
D-2-hydroxyglutaric aciduria in three patients with proven SSADH deficiency: genetic coincidence or a related biochemical epiphenomenon?
Breast Neoplasms
ADHFE1 is a MYC-linked oncogene that induces metabolic reprogramming and cellular de-differentiation in breast cancer.
Breast Neoplasms
Biochemical Background in Mitochondria Affects 2HG Production by IDH2 and ADHFE1 in Breast Carcinoma.
Carcinogenesis
Leptin Signaling Affects Survival and Chemoresistance of Estrogen Receptor Negative Breast Cancer.
Carcinoma
Identification and validation of highly frequent CpG island hypermethylation in colorectal adenomas and carcinomas.
Colonic Neoplasms
Identification of regulatory role of DNA methylation in colon cancer gene expression via systematic bioinformatics analysis.
Colorectal Neoplasms
Alcohol dehydrogenase, iron containing, 1 promoter hypermethylation associated with colorectal cancer differentiation.
Colorectal Neoplasms
Alcohol induces cell proliferation via hypermethylation of ADHFE1 in colorectal cancer cells.
Colorectal Neoplasms
Hypermethylation Of ADHFE1 Promotes The Proliferation Of Colorectal Cancer Cell Via Modulating Cell Cycle Progression.
Mouth Neoplasms
MiR-30a and miR-379 modulate retinoic acid pathway by targeting DNA methyltransferase 3B in oral cancer.
Neoplasms
Downregulation of HSP60 disrupts mitochondrial proteostasis to promote tumorigenesis and progression in clear cell renal cell carcinoma.
Neoplasms
Genome-wide DNA methylation profiles of low- and high-grade adenoma reveals potential biomarkers for early detection of colorectal carcinoma.
Stomach Neoplasms
Screening and Survival Analysis of Hub Genes in Gastric Cancer Based on Bioinformatics.
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KM VALUE [mM]
SUBSTRATE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
additional information
additional information
-
no activity with L-isomer of 2-hydroxybutyrate, Km for succinate semialdehyde is below 0.01 mM, 30°C, pH 7.1
-
0.077
2-oxoglutarate
-
+/-0.009 mM, in overexpressed HEK cells, in presence of 0.1 mM 4-hydroxybutyrate
0.067
4-hydroxybutyrate
-
+/-0.012 mM, in overepressed HEK cells, in presence of 0.5 mM 2-oxoglutarate
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Ki VALUE [mM]
INHIBITOR
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
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IC50 VALUE [mM]
INHIBITOR
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
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SPECIFIC ACTIVITY [µmol/min/mg]
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
additional information
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pH OPTIMUM
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
7.1
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TEMPERATURE OPTIMUM
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
30
37
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pI VALUE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
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ORGANISM
COMMENTARY
LITERATURE
UNIPROT
SEQUENCE DB
SOURCE
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SOURCE TISSUE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
SOURCE
-
of healthy subjects and patients with D-2-hydroxyglutaric aciduria
brenda
additional information
-
highest activity in liver and kidney and an intermediate activity in heart
brenda
-
highest activity in liver and kidney and an intermediate activity in heart
brenda
-
highest activity in liver and kidney and an intermediate activity in heart
brenda
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LOCALIZATION
ORGANISM
UNIPROT
COMMENTARY
GeneOntology No.
LITERATURE
SOURCE
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GENERAL INFORMATION
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
malfunction