Information on EC 1.14.11.1 - gamma-butyrobetaine dioxygenase

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The expected taxonomic range for this enzyme is: Eukaryota, Bacteria

EC NUMBER
COMMENTARY hide
1.14.11.1
-
RECOMMENDED NAME
GeneOntology No.
gamma-butyrobetaine dioxygenase
REACTION
REACTION DIAGRAM
COMMENTARY hide
ORGANISM
UNIPROT
LITERATURE
4-trimethylammoniobutanoate + 2-oxoglutarate + O2 = 3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
show the reaction diagram
REACTION TYPE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
oxidation
-
-
-
-
oxidative decarboxylation
-
-
-
-
redox reaction
-
-
-
-
reduction
-
-
-
-
PATHWAY
BRENDA Link
KEGG Link
MetaCyc Link
gamma-butyrobetaine degradation
-
-
L-carnitine biosynthesis
-
-
carnitine metabolism
-
-
Lysine degradation
-
-
SYSTEMATIC NAME
IUBMB Comments
4-trimethylammoniobutanoate,2-oxoglutarate:oxygen oxidoreductase (3-hydroxylating)
Requires Fe2+ and ascorbate.
CAS REGISTRY NUMBER
COMMENTARY hide
9045-31-2
-
ORGANISM
COMMENTARY hide
LITERATURE
UNIPROT
SEQUENCE DB
SOURCE
-
-
-
Manually annotated by BRENDA team
GENERAL INFORMATION
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
evolution
metabolism
physiological function
additional information
SUBSTRATE
PRODUCT                       
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate) hide
LITERATURE
(Substrate)
COMMENTARY
(Product) hide
LITERATURE
(Product)
Reversibility
r=reversible
ir=irreversible
?=not specified
(3-carboxypropyl)(trimethyl)arsanium + 2-oxoglutarate + O2
[(2R)-3-carboxy-2-hydroxypropyl](trimethyl)arsanium + succinate + CO2
show the reaction diagram
(3-carboxypropyl)(trimethyl)phosphanium + 2-oxoglutarate + O2
[(2R)-3-carboxy-2-hydroxypropyl](trimethyl)phosphanium + succinate + CO2
show the reaction diagram
(3R)-3-fluoro-4-(trimethylammonio)butanoate + 2-oxoglutarate + O2
3-carboxy-N,N,N-trimethyl-2-oxopropan-1-aminium + succinate + formylfluoride
show the reaction diagram
(3R)-3-fluoro-4-(trimethylammonio)butanoate + 2-oxoglutarate + O2
?
show the reaction diagram
-
weak substrate, below 5% turnover after 3 h
-
-
?
(3S)-3-fluoro-4-(trimethylammonio)butanoate + 2-oxoglutarate + O2
?
show the reaction diagram
-
S-stereoisomer preferred substrate
-
-
?
(R)-2-amino-3-carboxy-N,N,N-trimethylpropan-1-aminium + 2-oxoglutarate + O2
N-[(3R)-3-amino-3-carboxy-2-hydroxypropyl]-N,N-dimethylmethanaminium + succinate + CO2
show the reaction diagram
(S)-2-amino-3-carboxy-N,N,N-trimethylpropan-1-aminium + 2-oxoglutarate + O2
N-[(3S)-3-amino-3-carboxy-2-hydroxypropyl]-N,N-dimethylmethanaminium + succinate + CO2
show the reaction diagram
(S)-3-carboxy-2-hydroxy-N,N,N-trimethylpropan-1-aminium + 2-oxoglutarate + O2
(3S)-3-carboxy-2,3-dihydroxy-N,N,N-trimethylpropan-1-aminium + succinate + CO2
show the reaction diagram
-
-
-
-
?
2-carboxy-N,N,N-trimethylethan-1-aminium + 2-oxoglutarate + O2
4-carboxy-3-hydroxy-N,N,N-trimethylbutan-1-aminium + succinate + CO2
show the reaction diagram
3-carboxy-N-(fluoromethyl)-N,N-dimethylpropan-1-aminium + 2-oxoglutarate + O2
(2S)-3-carboxy-N-(fluoromethyl)-2-hydroxy-N,N-dimethylpropan-1-aminium + succinate + CO2
show the reaction diagram
3-trimethylaminopropionic acid + 2-oxoglutarate + O2
?
show the reaction diagram
-
20% of the hydroxylation rate of gamma-butyrobetaine
-
-
?
4-(trimethylammonio)butanoic acid + 2-oxoglutarate + O2
?
show the reaction diagram
-
-
-
-
?
4-dimethylaminobutyric acid + 2-oxoglutarate + O2
4-dimethylamino-3-hydroxybutyric acid + succinate + CO2
show the reaction diagram
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
show the reaction diagram
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
4-carboxy-3-hydroxy-N,N,N-trimethylbutan-1-aminium + succinate + CO2
show the reaction diagram
4-trimethylammoniopropanoate + 2-oxoglutarate + O2
2-carboxy-2-hydroxy-N,N,N-trimethylethan-1-aminium + succinate + CO2
show the reaction diagram
-
-
-
-
?
5,5-dimethylhexanoate + 2-oxoglutarate + O2
?
show the reaction diagram
6-N-trimethyl-L-lysine + 2-oxoglutarate + O2
?
show the reaction diagram
-
-
-
-
?
D-carnitine + 2-oxoglutarate + O2
3-carboxy-N,N,N-trimethyl-2-oxopropan-1-aminium + succinate + CO2
show the reaction diagram
gamma-butyrobetaine + 2-oxoglutarate + O2
carnitine + succinate + CO2
show the reaction diagram
L-carnitine + 2-oxoglutarate + O2
3-carboxy-N,N,N-trimethyl-2-oxopropan-1-aminium + succinate + CO2
show the reaction diagram
-
-
-
-
?
mildronate + O2
?
show the reaction diagram
-
both a relatively weak BBOX inhibitor in vitro and a competitive substrate producing multiple products
-
-
?
[(2R)-3-carboxy-2-hydroxypropyl](trimethyl)arsanium + 2-oxoglutarate + O2
(3-carboxy-2-oxopropyl)(trimethyl)arsanium + succinate + CO2
show the reaction diagram
[(2R)-3-carboxy-2-hydroxypropyl](trimethyl)phosphanium + 2-oxoglutarate + O2
(3-carboxy-2-oxopropyl)(trimethyl)phosphanium + succinate + CO2
show the reaction diagram
-
-
-
?
[(2S)-3-carboxy-2-hydroxypropyl](trimethyl)arsanium + 2-oxoglutarate + O2
(3-carboxy-2-oxopropyl)(trimethyl)arsanium + succinate + CO2
show the reaction diagram
-
-
-
?
[(2S)-3-carboxy-2-hydroxypropyl](trimethyl)phosphanium + 2-oxoglutarate + O2
(3-carboxy-2-oxopropyl)(trimethyl)phosphanium + succinate + CO2
show the reaction diagram
-
-
-
?
additional information
?
-
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
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
show the reaction diagram
additional information
?
-
COFACTOR
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
2,6-dichlorophenolindophenol
-
-
ascorbate
additional information
-
KCl, nicotinamide, MgCl2 or catalase not required for full activity
-
METALS and IONS
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
additional information
-
Ni2+ cannot substitute for Fe2+
INHIBITORS
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
(3-carboxy-2-oxopropyl)(trimethyl)arsanium
-
-
(3-carboxy-2-oxopropyl)(trimethyl)phosphanium
-
-
(3-carboxypropyl)(trimethyl)arsanium
-
-
(3-carboxypropyl)(trimethyl)phosphanium
-
-
(3R)-3-fluoro-4-(trimethylammonio)butanoate
-
-
(E)-4-(trimethylammonio)but-2-enoate
-
-
1,1,1-trimethyl-2-(2-phosphonoethyl)hydrazin-1-ium Iodide
-
-
1-(3-carboxypropyl)-1-methylpyrrolidin-1-ium
-
-
1-(3-carboxypropyl)pyrrolidin-1-ium
-
-
2-(1,1,1-trimethylhydrazin-1-ium-2-yl)ethanesulfonate
-
-
2-(2-carboxyethyl)-1,1-diethyl-1-methylhydrazin-1-ium chloride
-
-
2-(2-carboxyethyl)-1,1-dimethyl-1-(prop-2-yn-1-yl)-hydrazin-1-ium bromide
-
-
2-(2-carboxyethyl)-1-(2-chloroethyl)-1,1-dimethylhydrazin-1-ium bromide
-
-
2-oxoglutarate
-
at concentrations above 1 mM
3,4-dihydroxybenzoate
-
-
3-(1,1,1,2-tetramethylhydrazin-1-ium-2-yl)propanoate
-
-
3-(1,1,1-trimethylhydrazin-1-ium-2-yl)propanoate
3-(1,1,2-trimethyl-1-propylhydrazin-1-ium-2-yl)propanoate
-
-
3-(1,1-dimethyl-1-vinylhydrazin-1-ium-2-yl)propanoate
-
-
-
3-(1-allyl-1,1-dimethylhydrazin-1-ium-2-yl)propanoate
-
-
3-(1-ethyl-1,1,2-trimethylhydrazin-1-ium-2-yl)propanoate
-
-
3-(1-ethyl-1,1-dimethylhydrazin-1-ium-2-yl)propanoate
-
-
3-(1-isopropyl-1,1-dimethylhydrazin-1-ium-2-yl)propanoate
-
-
3-(2,2,2-trimethylhydrazinium propionate dihydrate)
-
i.e. mildronate, a synthetic inhibitor of GBBH, is a non-hydroxylatable analog of gamma-butyrobetaine
3-(2,2,2-trimethylhydrazinium)propionate
-
complete inhibition at 0.05 mM
3-(2,2,2-trimethylhydrazinium)propionate dihydrate
-
-
3-(2,2-Dimethylcyclopropyl)propionic acid
-
mechanism-based inhibitor
3-(2-ethyl-1,1,1-trimethylhydrazin-1-ium-2-yl)propanoate
-
-
3-(ethyldimethylammonio)propane-1-sulfonate
-
-
3-(trimethylammonio)cyclohexanecarboxylate
-
-
3-(trimethylammonio)propane-1-sulfonate
-
-
3-Bromo-2-oxoglutarate
-
noncompetitive inhibition, 2-oxoglutarate as variable substrate
3-carboxy-2-chloro-N,N,N-trimethylpropan-1-aminium
-
-
3-carboxy-N-(2-chloroethyl)-N,N-dimethylpropan-1-aminium
-
-
3-carboxy-N-(2-fluoroethyl)-N,N-dimethylpropan-1-aminium
-
-
3-carboxy-N-(fluoromethyl)-N,N-dimethylpropan-1-aminium
-
-
3-glutathione-2-oxoglutarate
-
noncompetitive to 2-oxoglutarate
3-hydroxy-N-(2-phenylethyl)pyridine-2-carboxamide
-
weak inhibition
3-trimethylaminopropyl-1-sulfonate
-
-
3-[1-(2-aminoethyl)-1,1-dimethylhydrazin-1-ium-2-yl]-propanoate
-
-
3-[1-(2-hydroxyethyl)-1,1-dimethylhydrazin-1-ium-2-yl]-propanoate
-
-
-
4-(allyldimethylammonio)butanoate
-
-
4-(benzyldimethylammonio)butanoate
-
-
4-(butyldimethylammonio)butanoate
-
-
4-(cyclobutyldimethylammonio)butanoate
-
-
4-(cyclopentyldimethylammonio)butanoate
-
-
4-(cyclopropyldimethylammonio)butanoate
-
-
4-(dimethylsulfonio)butanoate
-
-
4-(ethyldimethylammonio)butanoate
-
-
4-(trimethylammonio)pentanoate
-
-
4-Trimethylammoniobutanoate
4-[(2-hydroxyethyl)dimethylammonio]butanoate
-
-
4-[(bromomethyl)dimethylammonio]butanoate
-
-
4-[(chloromethyl)dimethylammonio]butanoate
-
-
4-[(cyclobutylmethyl)dimethylammonio]butanoate
-
-
4-[(cyclopropylmethyl)dimethylammonio]butanoate
-
-
4-[(iodomethyl)dimethylammonio]butanoate
-
-
4-[(methoxymethyl)dimethylammonio]butanoate
-
-
4-[diethyl(methyl)ammonio]butanoate
-
-
4-[dimethyl(prop-2-yn-1-yl)ammonio]butanoate
-
-
4-[dimethyl(propyl)ammonio]butanoate
-
-
4-[dimethyl(vinyl)ammonio]butanoate
-
-
4-[isopropyl(dimethyl)ammonio]butanoate
-
-
5-(trimethylammonio)pentanoate
-
-
alpha,alpha'-bipyridyl
-
no activity at 2 mM
AR692
-
-
AR692B
-
; a potent and selective inhibitor for human enzyme BBOX, binds in two modes, one of which adopts an unusual U-shape conformation stabilised by inter- and intra-molecular Pi-stacking interactions. Conformational changes observed on binding of the inhibitor to BBOX likely reflect those occurring in catalysis. The BBOX-AR692B with and without substrate/ inhibitor crystal structures (PDB ID 3O2G/3N6W) reveal substantial conformational differences
AR780
-
-
arsenite
-
-
ascorbate
Ba2+
-
-
benzeneselenenic acid
-
-
benzeneselenenyl bromide
-
-
benzeneselenenyl chloride
-
-
benzeneseleninic acid
-
-
Ca2+
-
-
Cd2+
-
-
Clofibrate
-
activity of gamma-butyrobetaine dioxygenase in the liver is lower in pigs treated with clofibrate than in control pigs
Co2+
-
-
Cu2+
-
-
cyclopropyl-substituted gamma-butyrobetaines
-
-
-
D-carnitine
-
uncoupling agent
dioxane
-
-
diphenyldiselane
-
-
DL-carnitine
ebselen
-
a relatively potent BBOX inhibitor and a Zn(II) ejector
FMN
-
in high concentrations
gamma-butyrobetaine
Hg2+
-
-
iodoacetate
-
less effective than p-chloromercuriphenylsulfonate
Iodosobenzoate
-
less effective than p-chloromercuriphenylsulfonate
Mg2+
-
-
mildronate
-
Mn2+
-
-
N,N,N-trimethyl-3-(1H-tetrazol-5-yl)propan-1-aminium
-
-
N,N,N-trimethyl-3-phosphonopropan-1-aminium
-
-
N-(1-chloro-4-hydroxyisoquinoline-3-carbonyl)-L-tryptophan
-
-
N-(1-chloro-4-hydroxyisoquinoline-3-carbonyl)glycine
-
-
N-(2-bromoethyl)-3-carboxy-N,N-dimethylpropan-1-aminium
-
-
N-(2-hydroxybenzoyl)-L-phenylalanine
-
-
N-(2-hydroxybenzoyl)glycine
-
weak inhibition
N-(3-hydroxypyridine-2-carbonyl)-beta-alanine
-
weak inhibition
N-(3-hydroxypyridine-2-carbonyl)-D-phenylalanine
-
weak inhibition
N-(3-hydroxypyridine-2-carbonyl)-L-glutamic acid
-
-
N-(3-hydroxypyridine-2-carbonyl)-L-phenylalanine
-
-
N-(3-hydroxypyridine-2-carbonyl)-S-[(pyridin-2-yl)methyl]-L-cysteine
-
-
N-(3-hydroxypyridine-2-carbonyl)glycine
-
; weak inhibition
N-(pyridine-2-carbonyl)-L-phenylalanine
-
-
N-ethylmaleimide
-
less effective than p-chloromercuriphenylsulfonate
N-oxalylglycine
N-[(1-chloro-4-hydroxy-3-isoquinolinyl)carbonyl]-glycine
-
; i.e. FG-2216, BIQ, or IOX3, an isoquinoline derivative, that inhibits BBOX and other 2-oxoglutarate dependent oxygenases through chelating active site Fe(II) and interacting with specific active site residues. Time course of Zn(II) ejection from BBOX by the inhibitors
O2
-
irreversible
p-aminomethylbenzoic acid
-
1.2 mmol/kg reduces the conversion of 4-dimethylaminobutyric acid to 4-dimethylamino-3-hydroxybutyric acid from 62.6% to 46.8%
p-chloromercuribenzoate
-
inactivates enzyme completely at 0.1 mM
p-Chloromercuriphenylsulfonate
-
inactivates enzyme completely at 0.1 mM
Pb2+
-
-
phosphate
-
-
Pyridine 2,4-dicarboxylate
-
-
Pyridine-2,4-dicarboxylate
-
-
Quinacrine
-
-
Sn2+
-
-
structure analogues of gamma-butyrobetaine and 2-oxoglutarate
-
Succinic semialdehyde
-
-
thiram
-
-
Zn2+
-
-
ZnCl2
-
1 mM, complete inhibition
[(2R)-3-carboxy-2-hydroxypropyl](trimethyl)arsanium
-
-
[(2R)-3-carboxy-2-hydroxypropyl](trimethyl)phosphanium
-
-
[(2S)-3-carboxy-2-hydroxypropyl](trimethyl)arsanium
-
-
[(2S)-3-carboxy-2-hydroxypropyl](trimethyl)phosphanium
-
-
[3-(ethyldimethylammonio)propyl]phosphinate
-
-
[3-(trimethylammonio)propyl]phosphinate
-
-
additional information
-
ACTIVATING COMPOUND
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
2-amino-6,7-dimethyl-4-hydroxy-5,6,7,8-tetrahydropteridine
-
-
ascorbate
catalase
-
Cs+
-
increase of activity
GSH/GSH-peroxidase
-
increase of activity, more efficient in assay and during preincubation than catalase, protects the enzyme from increasing phosphate concentrations
-
isoascorbate
-
increase of activity
K+
-
efficient coupling of decarboxylation and hydroxylation, stimulation
L-ascorbate
-
required
L-histidine
-
-
Microsomal preparation
-
increase of activity
-
NADPH
-
NADPH-regenerating system, increase of activity, no absolute requirement
NH4+
-
,increase of activity
nicotinamide
-
increase of activity
Rb+
-
increase of activity
additional information
-
various proteins such as lactoperoxidase, horseradish peroxidase, hemoglobin, myoglobin, cytochrome c, bovine serum albumin activate
-
KM VALUE [mM]
SUBSTRATE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
0.02 - 0.412
(2S)-3-carboxy-N-(fluoromethyl)-2-hydroxy-N,N-dimethylpropan-1-aminium
1.37
(3-carboxypropyl)(trimethyl)arsanium
-
pH 7.5, 23°C
1.2
(3-carboxypropyl)(trimethyl)phosphanium
-
pH 7.5, 23°C
0.02 - 0.623
(3R)-3-fluoro-4-(trimethylammonio)butanoate
0.012 - 0.083
(3S)-3-fluoro-4-(trimethylammonio)butanoate
0.125
2-oxo-glutarate
-
-
0.018 - 0.82
2-oxoglutarate
0.16
4-(trimethylammonio)butanoic acid
-
co-substrate: 2-oxoglutarate, pH 7.5, 25°C, NMR assay
0.04
4-Dimethylaminobutyric acid
-
in 20mM potassium phosphate buffer (pH 7.4) containing 50 mM KCl, 3mM 2-oxoglutarate, 10 mM sodium ascorbate, 0.5 mM DTT, 0.5 mM ammonium iron sulfate, 2.5 mg/ml bovine serum albumin, 0.01% (w/v) Triton X-100 and 2 mM 6-N-trimethyllysine
0.0042 - 2.474
4-Trimethylammoniobutanoate
0.47
D-carnitine
-
-
0.012
Fe2+
-
-
0.029 - 2.4
gamma-butyrobetaine
0.052
L-carnitine
-
-
additional information
additional information
-
TURNOVER NUMBER [1/s]
SUBSTRATE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
0.3 - 5
(2S)-3-carboxy-N-(fluoromethyl)-2-hydroxy-N,N-dimethylpropan-1-aminium
2.61
(3-carboxypropyl)(trimethyl)arsanium
-
pH 7.5, 23°C
4.13
(3-carboxypropyl)(trimethyl)phosphanium
-
pH 7.5, 23°C
0.059 - 0.14
(3R)-3-fluoro-4-(trimethylammonio)butanoate
0.1 - 0.62
(3S)-3-fluoro-4-(trimethylammonio)butanoate
0.39 - 5.1
2-oxoglutarate
5.3
4-(trimethylammonio)butanoic acid
-
co-substrate: 2-oxoglutarate, pH 7.5, 25°C, NMR assay
0.83 - 21.3
4-Trimethylammoniobutanoate
34
gamma-butyrobetaine
-
-
Ki VALUE [mM]
INHIBITOR
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
0.136
(3R)-3-fluoro-4-(trimethylammonio)butanoate
-
pH 7.5, temperature not specified in the publication
0.0006
3,4-dihydroxybenzoate
-
-
0.016
3-(1,1,1-trimethylhydrazin-1-ium-2-yl)propanoate
-
pH 7.0, 37°C, recombinant enzyme
0.226
3-carboxy-N-(fluoromethyl)-N,N-dimethylpropan-1-aminium
-
pH 7.5, temperature not specified in the publication
0.024 - 0.125
4-Trimethylammoniobutanoate
0.0002
Pyridine-2,4-dicarboxylate
-
-
IC50 VALUE [mM]
INHIBITOR
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
0.0038
(E)-4-(trimethylammonio)but-2-enoate
Homo sapiens;
-
pH 7.0, 37°C, recombinant enzyme
0.117
1,1,1-trimethyl-2-(2-phosphonoethyl)hydrazin-1-ium Iodide
Homo sapiens;
-
pH 7.0, 37°C, recombinant enzyme
0.00049
1-(3-carboxypropyl)-1-methylpyrrolidin-1-ium
Homo sapiens;
-
pH 7.0, 37°C, recombinant enzyme
1
1-(3-carboxypropyl)pyrrolidin-1-ium
Homo sapiens;
-
pH 7.0, 37°C, recombinant enzyme
0.087
2-(1,1,1-trimethylhydrazin-1-ium-2-yl)ethanesulfonate
Homo sapiens;
-
pH 7.0, 37°C, recombinant enzyme
1
2-(2-carboxyethyl)-1,1-diethyl-1-methylhydrazin-1-ium chloride
Homo sapiens;
-
pH 7.0, 37°C, recombinant enzyme
0.024
2-(2-carboxyethyl)-1,1-dimethyl-1-(prop-2-yn-1-yl)-hydrazin-1-ium bromide
Homo sapiens;
-
pH 7.0, 37°C, recombinant enzyme
0.0102
2-(2-carboxyethyl)-1-(2-chloroethyl)-1,1-dimethylhydrazin-1-ium bromide
Homo sapiens;
-
pH 7.0, 37°C, recombinant enzyme
0.00009
3-(1,1,1,2-tetramethylhydrazin-1-ium-2-yl)propanoate
Homo sapiens;
-
pH 7.0, 37°C, recombinant enzyme
0.0031
3-(1,1,2-trimethyl-1-propylhydrazin-1-ium-2-yl)propanoate
Homo sapiens;
-
pH 7.0, 37°C, recombinant enzyme
0.0068
3-(1,1-dimethyl-1-vinylhydrazin-1-ium-2-yl)propanoate
Homo sapiens;
-
pH 7.0, 37°C, recombinant enzyme
-
0.119
3-(1-allyl-1,1-dimethylhydrazin-1-ium-2-yl)propanoate
Homo sapiens;
-
pH 7.0, 37°C, recombinant enzyme
0.0072
3-(1-ethyl-1,1,2-trimethylhydrazin-1-ium-2-yl)propanoate
Homo sapiens;
-
pH 7.0, 37°C, recombinant enzyme
0.14
3-(1-ethyl-1,1-dimethylhydrazin-1-ium-2-yl)propanoate
Homo sapiens;
-
pH 7.0, 37°C, recombinant enzyme
1
3-(1-isopropyl-1,1-dimethylhydrazin-1-ium-2-yl)propanoate
Homo sapiens;
-
pH 7.0, 37°C, recombinant enzyme
0.062
3-(2,2,2-trimethylhydrazinium propionate dihydrate)
Homo sapiens;
-
pH 7.0, 37°C, recombinant enzyme
0.0028
3-(2-ethyl-1,1,1-trimethylhydrazin-1-ium-2-yl)propanoate
Homo sapiens;
-
pH 7.0, 37°C, recombinant enzyme
0.0067
3-(ethyldimethylammonio)propane-1-sulfonate
Homo sapiens;
-
pH 7.0, 37°C, recombinant enzyme
1
3-(trimethylammonio)cyclohexanecarboxylate
Homo sapiens;
-
pH 7.0, 37°C, recombinant enzyme
0.0017
3-(trimethylammonio)propane-1-sulfonate
Homo sapiens;
-
pH 7.0, 37°C, recombinant enzyme
0.459
3-carboxy-2-chloro-N,N,N-trimethylpropan-1-aminium
Homo sapiens;
-
pH 7.0, 37°C, recombinant enzyme
0.00026
3-carboxy-N-(2-chloroethyl)-N,N-dimethylpropan-1-aminium
Homo sapiens;
-
pH 7.0, 37°C, recombinant enzyme
0.00061
3-carboxy-N-(2-fluoroethyl)-N,N-dimethylpropan-1-aminium
Homo sapiens;
-
pH 7.0, 37°C, recombinant enzyme
1
3-hydroxy-N-(2-phenylethyl)pyridine-2-carboxamide
Homo sapiens;
-
above, pH and temperature not specified in the publication
1
3-[1-(2-aminoethyl)-1,1-dimethylhydrazin-1-ium-2-yl]-propanoate
Homo sapiens;
-
pH 7.0, 37°C, recombinant enzyme
1
3-[1-(2-hydroxyethyl)-1,1-dimethylhydrazin-1-ium-2-yl]-propanoate
Homo sapiens;
-
pH 7.0, 37°C, recombinant enzyme
-
0.0027
4-(allyldimethylammonio)butanoate
Homo sapiens;
-
pH 7.0, 37°C, recombinant enzyme
1
4-(benzyldimethylammonio)butanoate
Homo sapiens;
-
pH 7.0, 37°C, recombinant enzyme
1
4-(butyldimethylammonio)butanoate
Homo sapiens;
-
pH 7.0, 37°C, recombinant enzyme
0.00081
4-(cyclobutyldimethylammonio)butanoate
Homo sapiens;
-
pH 7.0, 37°C, recombinant enzyme
1
4-(cyclopentyldimethylammonio)butanoate
Homo sapiens;
-
pH 7.0, 37°C, recombinant enzyme
0.0021
4-(cyclopropyldimethylammonio)butanoate
Homo sapiens;
-
pH 7.0, 37°C, recombinant enzyme
0.388
4-(dimethylsulfonio)butanoate
Homo sapiens;
-
pH 7.0, 37°C, recombinant enzyme
0.0033
4-(ethyldimethylammonio)butanoate
Homo sapiens;
-
pH 7.0, 37°C, recombinant enzyme
0.343
4-(trimethylammonio)pentanoate
Homo sapiens;
-
pH 7.0, 37°C, recombinant enzyme
1
4-[(2-hydroxyethyl)dimethylammonio]butanoate
Homo sapiens;
-
pH 7.0, 37°C, recombinant enzyme
1
4-[(bromomethyl)dimethylammonio]butanoate
Homo sapiens;
-
pH 7.0, 37°C, recombinant enzyme
0.268
4-[(chloromethyl)dimethylammonio]butanoate
Homo sapiens;
-
pH 7.0, 37°C, recombinant enzyme
1
4-[(cyclobutylmethyl)dimethylammonio]butanoate
Homo sapiens;
-
pH 7.0, 37°C, recombinant enzyme
1
4-[(cyclopropylmethyl)dimethylammonio]butanoate
Homo sapiens;
-
pH 7.0, 37°C, recombinant enzyme
1
4-[(iodomethyl)dimethylammonio]butanoate
Homo sapiens;
-
pH 7.0, 37°C, recombinant enzyme
1
4-[(methoxymethyl)dimethylammonio]butanoate
Homo sapiens;
-
pH 7.0, 37°C, recombinant enzyme
0.1
4-[diethyl(methyl)ammonio]butanoate
Homo sapiens;
-
pH 7.0, 37°C, recombinant enzyme
0.0014
4-[dimethyl(prop-2-yn-1-yl)ammonio]butanoate
Homo sapiens;
-
pH 7.0, 37°C, recombinant enzyme
1
4-[dimethyl(propyl)ammonio]butanoate
Homo sapiens;
-
pH 7.0, 37°C, recombinant enzyme
0.127
4-[dimethyl(vinyl)ammonio]butanoate
Homo sapiens;
-
pH 7.0, 37°C, recombinant enzyme
0.0057
4-[isopropyl(dimethyl)ammonio]butanoate
Homo sapiens;
-
pH 7.0, 37°C, recombinant enzyme
1
5-(trimethylammonio)pentanoate
Homo sapiens;
-
pH 7.0, 37°C, recombinant enzyme
0.000153
AR692B
Homo sapiens;
-
pH and temperature not specified in the publication
0.019
benzeneselenenic acid
Homo sapiens;
-
pH 7.0, 22°C, 20 min
0.003
benzeneselenenyl bromide
Homo sapiens;
-
pH 7.0, 22°C, 20 min
0.0029
benzeneselenenyl chloride
Homo sapiens;
-
pH 7.0, 22°C, 20 min
0.0047
benzeneseleninic acid
Homo sapiens;
-
pH 7.0, 22°C, 20 min
0.042
diphenyldiselane
Homo sapiens;
-
pH 7.0, 22°C, 20 min
0.00083
ebselen
Homo sapiens;
-
pH 7.0, 22°C, 20 min
0.06
mildronate
Homo sapiens;
-
pH and temperature not specified in the publication
-
1
N,N,N-trimethyl-3-(1H-tetrazol-5-yl)propan-1-aminium
Homo sapiens;
-
pH 7.0, 37°C, recombinant enzyme
0.0001
N,N,N-trimethyl-3-phosphonopropan-1-aminium
Homo sapiens;
-
pH 7.0, 37°C, recombinant enzyme
0.026
N-(1-chloro-4-hydroxyisoquinoline-3-carbonyl)-L-tryptophan
Homo sapiens;
-
pH 7.5, temperature not specified in the publication, recombinant enzyme
0.0012 - 0.018
N-(1-chloro-4-hydroxyisoquinoline-3-carbonyl)glycine
0.0012
N-(2-bromoethyl)-3-carboxy-N,N-dimethylpropan-1-aminium
Homo sapiens;
-
pH 7.0, 37°C, recombinant enzyme
0.088
N-(2-hydroxybenzoyl)-L-phenylalanine
Homo sapiens;
-
pH and temperature not specified in the publication
0.537
N-(2-hydroxybenzoyl)glycine
Homo sapiens;
-
pH and temperature not specified in the publication
0.522
N-(3-hydroxypyridine-2-carbonyl)-beta-alanine
Homo sapiens;
-
pH and temperature not specified in the publication
0.15
N-(3-hydroxypyridine-2-carbonyl)-D-phenylalanine
Homo sapiens;
-
pH and temperature not specified in the publication
0.0018
N-(3-hydroxypyridine-2-carbonyl)-L-glutamic acid
Homo sapiens;
-
pH and temperature not specified in the publication
0.0005 - 0.002
N-(3-hydroxypyridine-2-carbonyl)-L-phenylalanine
0.006 - 1
N-(3-hydroxypyridine-2-carbonyl)glycine
0.0125
N-(pyridine-2-carbonyl)-L-phenylalanine
Homo sapiens;
-
pH and temperature not specified in the publication
0.032
N-[(1-chloro-4-hydroxy-3-isoquinolinyl)carbonyl]-glycine
Homo sapiens;
-
pH 7.0, 22°C, 20 min
0.082
Pyridine 2,4-dicarboxylate
Homo sapiens;
-
pH 7.5, 25°C
0.0079
thiram
Homo sapiens;
-
pH 7.0, 22°C, 20 min
0.00053
[3-(ethyldimethylammonio)propyl]phosphinate
Homo sapiens;
-
pH 7.0, 37°C, recombinant enzyme
0.00052
[3-(trimethylammonio)propyl]phosphinate
Homo sapiens;
-
pH 7.0, 37°C, recombinant enzyme
SPECIFIC ACTIVITY [µmol/min/mg]
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
0.00000125
-
value below, activity in heart; value below, activity in skeletal muscle
0.000008
-
activity in spleen
0.000018
-
activity in brain
0.00007
-
activity in ileum
0.000393
-
activity in liver
0.0004
-
activity of gamma-butyrobetaine dioxygenase in liver
0.002367
-
activity in kidney
0.0024
-
activity of gamma-butyrobetaine dioxygenase in kidney
0.053
-
-
0.162
-
human kidney
1.02 - 1.74
-
three isozymes of mammalian enzyme
additional information
pH OPTIMUM
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
pH RANGE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
6 - 7.5
-
half-maximal activity at pH 6.0 and 7.5
6 - 8.4
-
half-maximal activity at pH 6.0 and 8.4, partially purified preparation
TEMPERATURE OPTIMUM
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
22
-
assay at room temperature
23
-
assay at
25
-
assay at
SOURCE TISSUE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
SOURCE
-
-
Manually annotated by BRENDA team
-
-
Manually annotated by BRENDA team
additional information
-
no activity found: muscle, kidney
Manually annotated by BRENDA team
PDB
SCOP
CATH
UNIPROT
ORGANISM
Homo sapiens;
MOLECULAR WEIGHT
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
37000
-
1 * 39000 + 1 * 37000, SDS-PAGE, amino acid analysis, N-terminal amino acid sequence
39000
-
1 * 39000 + 1 * 37000, SDS-PAGE, amino acid analysis, N-terminal amino acid sequence
44000
-
x * 44000, SDS-PAGE
46000
-
2 * 46000, SDS-PAGE
64000
-
gel filtration, 3 isozymes, detection by chromatofocusing and anion exchange chromatography
68000
-
gel filtration
80000
-
gel filtration
86000
-
gel filtration
86000 - 95000
-
gel filtration, sedimentation equilibrium centrifugation
SUBUNITS
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
homodimer
additional information
Crystallization/COMMENTARY
ORGANISM
UNIPROT
LITERATURE
analysis of a crystal structure of human BBOX in complex with inhibitor AR692B and Ni2+ (an Fe2+ surrogate), P212121 space group, 2.8 A resolution
-
crystal structure analysis, PDB ID 3O2G
-
purified recombinant enzyme in complex with inhibitors 4-(ethyldimethylammonio)butanoate, 3-carboxy-N-(2-fluoroethyl)-N,N-dimethylpropan-1-aminium, 1-(3-carboxypropyl)-1-methylpyrrolidin-1-ium, 4-(trimethylammonio)pentanoate, [3-(trimethylammonio)propyl]phosphinate, and 3-(1,1,1,2-tetramethylhydrazin-1-ium-2-yl)propanoate, sitting drop vapor technique, mixing of 0.001 ml of 7 mg/ml protein, 8 mM N-oxalylglycine and 4 mM inhibitor, with 0.001 ml of well solution containing 0.2 M ammonium citrate, 20% PEG 3350, 3% hexanediamine, and 10 mM ZnSO4, pH 7.0, X-ray diffraction structure determination and analysis at 1.70-2.40 A resolution
-
purified recombinant His-tagged GBBH, and SeMet-labeled variant, by sitting drop vapor diffusion technique, mixing 0.001 ml of 7 mg/ml protein in 20 mM Tris-HCl, pH 8.0, with 0.001 ml of reservoir solution containing 1.2 M ammonium sulfate and 100 mM sodium acetate, pH 4.5. In co-crystallization trials with ligands, each ligand is added to the reservoir solution to a final concentration of 5 mM, X-ray diffraction structure determination and analysis at 2.0 A and 3.5 A resolution, respectively
-
TEMPERATURE STABILITY
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
50
-
12 min half-life
60
-
complete inactivation after 15 min
GENERAL STABILITY
ORGANISM
UNIPROT
LITERATURE
dialysis, 4°C, 50% loss of activity overnight
-
DTT, prevents inactivation by O2, ascorbate or H2O2
-
His, stabilizes
-
ultra-filtration, 4°C, 50% loss of activity overnight
-
ORGANIC SOLVENT
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
Acetone
-
powder, retains full activity of fresh liver homogenate
OXIDATION STABILITY
ORGANISM
UNIPROT
LITERATURE
O2, irreversible inactivation during preincubation
STORAGE STABILITY
ORGANISM
UNIPROT
LITERATURE
-20°C to 4°C, 50% loss of activity in a week
-
-60° to -170°C, stable for 6 months in potassium phosphate buffer, pH 6.5
-
-90°C, stable for a year or more as acetone powder, stable for 3 months as purified and lyophilized enzyme
-
4°C, 50 mM sodium phosphate pH 7.4, 20 mM KCl, 10 mM DTT, 200g/l glycerol are best conservation conditions
-
addition of EDTA causes total loss of activity
-
Purification/COMMENTARY
ORGANISM
UNIPROT
LITERATURE
recombinant His-tagged enzyme by nickel affinity chromatography, gel filtration, ultrafiltration, and buffer exchange via desalting gel filtration
-
recombinant His-tagged enzyme from Escherichia coli strain BL21(DE3) by nickel affinity chromatography, gel filtration, and anion exchange chromatography
-
recombinant His-tagged enzyme from Escherichia coli strain BL21(DE3) by nickel affinity chromatography, gel filtration, and anion exchange chromatography, to over 90% purity
-
recombinant His-tagged GBBH from Escherichia coli strain WK6 by nickel affinity chromatography
-
recombinant His6-tagged enzyme from Saccharomyces cerevisiae strain AH22 by nickel affinity chromatography, desalting gel filtration, anion exchange chromatography, tag cleavage by recombinant TEV protease, desalting gel filtration, and another step of nickel affinity chromatography to remove the His-tag
-
Cloned/COMMENTARY
ORGANISM
UNIPROT
LITERATURE
expression in HEK293 cells
-
expression of His-tagged GBBH in Escherichia coli strain WK6
-
gene BBOX1, recombinant expression of His-tagged codon-optimized enzyme in Escherichia coli strain BL21(DE3)
-
recombinant expression of His-tagged enzyme
-
recombinant expression of His6-tagged enzyme in Saccharomyces cerevisiae strain AH22
-
recombinant expression of N-terminally His-tagged codon-optimized enzyme in Escherichia coli strain BL21(DE3)
-
recombinant protein is used
-
EXPRESSION
ORGANISM
UNIPROT
LITERATURE
activity of gamma-butyrobetaine dioxygenase lowest at birth in liver and kidney, but increase until age of 2 weeks
-
detection of mRNA levels during suckling period onwards, expression is ontogenically regulated
highest mRNA concentrations in epididymis and in kidney. In heart, colon, small intestine, lung, spleen, testes and brain lower mRNA levels compared to liver. In skeletal muscle almost absent mRNA amount
-
APPLICATION
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
drug development
-
enzyme BBOX is a drug target for the treatment of myocardial infarction
medicine
molecular biology
-
a fluorescence assay based on the detection of fluoride released from reactions of fluorinated substrates with BBOX by the use of tert-butyldimethylsilyl-protected fluorescein is developed. (3S)-3-fluoro-4-(trimethylammonio)butanoate is a good substrate for BBOX, releasing fluoride when subjected to the enzyme
Show AA Sequence (186 entries)
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