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Information on EC 1.14.11.1 - gamma-butyrobetaine dioxygenase
for references in articles please use BRENDA:EC1.14.11.1
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EC Tree 1 Oxidoreductases
1.14 Acting on paired donors, with incorporation or reduction of molecular oxygen
1.14.11 With 2-oxoglutarate as one donor, and incorporation of one atom of oxygen into each donor
1.14.11.1 gamma-butyrobetaine dioxygenase
IUBMB Comments
Requires Fe2+ and ascorbate.
The expected taxonomic range for this enzyme is: Eukaryota, Bacteria
- 1.14.11.1
-
cardioprotective
-
carnitine-dependent
-
3-2,2,2-trimethylhydrazinium
- mildronate
- trimethyllysine
- mid1
-
alpha4
- medicine
- d-carnitine
- dihydrate
- drug development
- molecular biology
Reaction Schemes
showSynonyms
gamma-butyrobetaine hydroxylase, bbox1, butyrobetaine hydroxylase, gamma-butyrobetaine dioxygenase, gamma-butyrobetaine hydroxylase 1, bu hydroxylase, gbb hydroxylase, alpha-butyrobetaine hydroxylase, more
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SYNONYM 
ORGANISM
UNIPROT
COMMENTARY 
LITERATURE
alpha-butyrobetaine hydroxylase
-
-
-
-
BBD
BBH
BBOX
BBOX1
butyrobetaine hydroxylase
-
-
-
-
gamma-butyrobetaine hydroxylase
oxygenase, gamma-butyrobetaine di-
-
-
-
-
psBBOX
gamma-butyrobetaine hydroxylase
-
-
-
-
gamma-butyrobetaine hydroxylase
-
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REACTION
REACTION DIAGRAM
COMMENTARY
ORGANISM
UNIPROT
LITERATURE
4-trimethylammoniobutanoate + 2-oxoglutarate + O2 = 3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
4-trimethylammoniobutanoate + 2-oxoglutarate + O2 = 3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
srereochemistry of reaction, pro-R hydrogen atom abstraction
-
4-trimethylammoniobutanoate + 2-oxoglutarate + O2 = 3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
srereochemistry of reaction, pro-R hydrogen atom abstraction
-
4-trimethylammoniobutanoate + 2-oxoglutarate + O2 = 3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
srereochemistry of reaction, pro-R hydrogen atom abstraction
-
-
4-trimethylammoniobutanoate + 2-oxoglutarate + O2 = 3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
-
-
-
-
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REACTION TYPE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
oxidation
-
-
-
-
oxidative decarboxylation
-
-
-
-
redox reaction
-
-
-
-
reduction
-
-
-
-
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PATHWAY SOURCE
PATHWAYS
BRENDA
KEGG
MetaCyc
gamma-butyrobetaine degradation II, L-carnitine biosynthesis
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SYSTEMATIC NAME
IUBMB Comments
4-trimethylammoniobutanoate,2-oxoglutarate:oxygen oxidoreductase (3-hydroxylating)
Requires Fe2+ and ascorbate.
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CAS REGISTRY NUMBER
COMMENTARY
9045-31-2
-
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SUBSTRATE
PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
LITERATURE
COMMENTARY
Reversibility
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
(3-carboxypropyl)(trimethyl)arsanium + 2-oxoglutarate + O2
[(2R)-3-carboxy-2-hydroxypropyl](trimethyl)arsanium + succinate + CO2
(3-carboxypropyl)(trimethyl)phosphanium + 2-oxoglutarate + O2
[(2R)-3-carboxy-2-hydroxypropyl](trimethyl)phosphanium + succinate + CO2
(3R)-3-fluoro-4-(trimethylammonio)butanoate + 2-oxoglutarate + O2
3-carboxy-N,N,N-trimethyl-2-oxopropan-1-aminium + succinate + formylfluoride
(3R)-3-fluoro-4-(trimethylammonio)butanoate + 2-oxoglutarate + O2
?
-
Substrates: weak substrate, below 5% turnover after 3 h
Products: -
Products: -
?
(3S)-3-fluoro-4-(trimethylammonio)butanoate + 2-oxoglutarate + O2
?
-
Substrates: S-stereoisomer preferred substrate
Products: -
Products: -
?
(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
(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
(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
Substrates: -
Products: -
Products: -
?
2-carboxy-N,N,N-trimethylethan-1-aminium + 2-oxoglutarate + O2
4-carboxy-3-hydroxy-N,N,N-trimethylbutan-1-aminium + succinate + CO2
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
3-trimethylaminopropionic acid + 2-oxoglutarate + O2
?
-
Substrates: 20% of the hydroxylation rate of gamma-butyrobetaine
Products: -
Products: -
?
4-(trimethylammonio)butanoic acid + 2-oxoglutarate + O2
?
-
Substrates: -
Products: -
Products: -
?
4-dimethylaminobutyric acid + 2-oxoglutarate + O2
4-dimethylamino-3-hydroxybutyric acid + succinate + CO2
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
4-trimethylammoniopropanoate + 2-oxoglutarate + O2
2-carboxy-2-hydroxy-N,N,N-trimethylethan-1-aminium + succinate + CO2
Substrates: -
Products: -
Products: -
?
D-carnitine + 2-oxoglutarate + O2
3-carboxy-N,N,N-trimethyl-2-oxopropan-1-aminium + succinate + CO2
L-carnitine + 2-oxoglutarate + O2
3-carboxy-N,N,N-trimethyl-2-oxopropan-1-aminium + succinate + CO2
mildronate + O2
?
Substrates: both a relatively weak BBOX inhibitor in vitro and a competitive substrate producing multiple products
Products: -
Products: -
?
[(2R)-3-carboxy-2-hydroxypropyl](trimethyl)arsanium + 2-oxoglutarate + O2
(3-carboxy-2-oxopropyl)(trimethyl)arsanium + succinate + CO2
[(2R)-3-carboxy-2-hydroxypropyl](trimethyl)phosphanium + 2-oxoglutarate + O2
(3-carboxy-2-oxopropyl)(trimethyl)phosphanium + succinate + CO2
Substrates: -
Products: -
Products: -
?
[(2S)-3-carboxy-2-hydroxypropyl](trimethyl)arsanium + 2-oxoglutarate + O2
(3-carboxy-2-oxopropyl)(trimethyl)arsanium + succinate + CO2
Substrates: -
Products: -
Products: -
?
[(2S)-3-carboxy-2-hydroxypropyl](trimethyl)phosphanium + 2-oxoglutarate + O2
(3-carboxy-2-oxopropyl)(trimethyl)phosphanium + succinate + CO2
Substrates: -
Products: -
Products: -
?
(3-carboxypropyl)(trimethyl)arsanium + 2-oxoglutarate + O2
[(2R)-3-carboxy-2-hydroxypropyl](trimethyl)arsanium + succinate + CO2
Substrates: -
Products: -
Products: -
?
(3-carboxypropyl)(trimethyl)arsanium + 2-oxoglutarate + O2
[(2R)-3-carboxy-2-hydroxypropyl](trimethyl)arsanium + succinate + CO2
Substrates: -
Products: -
Products: -
?
(3-carboxypropyl)(trimethyl)phosphanium + 2-oxoglutarate + O2
[(2R)-3-carboxy-2-hydroxypropyl](trimethyl)phosphanium + succinate + CO2
Substrates: -
Products: -
Products: -
?
(3-carboxypropyl)(trimethyl)phosphanium + 2-oxoglutarate + O2
[(2R)-3-carboxy-2-hydroxypropyl](trimethyl)phosphanium + succinate + CO2
Substrates: -
Products: -
Products: -
?
(3R)-3-fluoro-4-(trimethylammonio)butanoate + 2-oxoglutarate + O2
3-carboxy-N,N,N-trimethyl-2-oxopropan-1-aminium + succinate + formylfluoride
Substrates: -
Products: -
Products: -
?
(3R)-3-fluoro-4-(trimethylammonio)butanoate + 2-oxoglutarate + O2
3-carboxy-N,N,N-trimethyl-2-oxopropan-1-aminium + succinate + formylfluoride
Substrates: -
Products: -
Products: -
?
(3R)-3-fluoro-4-(trimethylammonio)butanoate + 2-oxoglutarate + O2
3-carboxy-N,N,N-trimethyl-2-oxopropan-1-aminium + succinate + formylfluoride
Substrates: -
Products: -
Products: -
?
(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
Substrates: -
Products: -
Products: -
?
(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
Substrates: -
Products: -
Products: -
?
(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
Substrates: -
Products: -
Products: -
?
(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
Substrates: -
Products: -
Products: -
?
2-carboxy-N,N,N-trimethylethan-1-aminium + 2-oxoglutarate + O2
4-carboxy-3-hydroxy-N,N,N-trimethylbutan-1-aminium + succinate + CO2
Substrates: -
Products: -
Products: -
?
2-carboxy-N,N,N-trimethylethan-1-aminium + 2-oxoglutarate + O2
4-carboxy-3-hydroxy-N,N,N-trimethylbutan-1-aminium + succinate + CO2
Substrates: -
Products: -
Products: -
?
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
Substrates: -
Products: -
Products: -
?
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
Substrates: -
Products: -
Products: -
?
4-dimethylaminobutyric acid + 2-oxoglutarate + O2
4-dimethylamino-3-hydroxybutyric acid + succinate + CO2
-
Substrates: -
Products: -
Products: -
?
4-dimethylaminobutyric acid + 2-oxoglutarate + O2
4-dimethylamino-3-hydroxybutyric acid + succinate + CO2
-
Substrates: -
Products: -
Products: -
?
4-dimethylaminobutyric acid + 2-oxoglutarate + O2
4-dimethylamino-3-hydroxybutyric acid + succinate + CO2
-
Substrates: poor substrate
Products: -
Products: -
?
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
-
Substrates: -
Products: -
Products: -
?
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
-
Substrates: gamma-butyrobetaine, terminal reaction in the pathway of L-carnitine biosynthesis
Products: -
Products: -
?
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
-
Substrates: -
Products: -
Products: -
?
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
-
Substrates: -
Products: -
Products: -
?
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
Substrates: -
Products: -
Products: -
?
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
Substrates: catalytic domain and active site structure, overview
Products: -
Products: -
?
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
-
Substrates: -
Products: -
Products: -
?
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
Substrates: -
Products: -
Products: -
?
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
Substrates: -
Products: -
Products: -
?
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
-
Substrates: -
Products: -
Products: -
?
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
-
Substrates: -
Products: -
Products: -
?
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
-
Substrates: i.e. gamma-butyrobetaine
Products: i.e. L-carnitine
Products: i.e. L-carnitine
?
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
Substrates: -
Products: -
Products: -
?
5,5-dimethylhexanoate + 2-oxoglutarate + O2
?
-
Substrates: poor substrate, 20% of the decarboxylation events lead to hydroxylation
Products: -
Products: -
?
5,5-dimethylhexanoate + 2-oxoglutarate + O2
?
-
Substrates: poor substrate, 20% of the decarboxylation events lead to hydroxylation
Products: -
Products: -
?
D-carnitine + 2-oxoglutarate + O2
3-carboxy-N,N,N-trimethyl-2-oxopropan-1-aminium + succinate + CO2
Substrates: -
Products: -
Products: -
?
D-carnitine + 2-oxoglutarate + O2
3-carboxy-N,N,N-trimethyl-2-oxopropan-1-aminium + succinate + CO2
Substrates: -
Products: -
Products: -
?
D-carnitine + 2-oxoglutarate + O2
3-carboxy-N,N,N-trimethyl-2-oxopropan-1-aminium + succinate + CO2
-
Substrates: -
Products: -
Products: -
r
gamma-butyrobetaine + 2-oxoglutarate + O2
carnitine + succinate + CO2
-
Substrates: -
Products: -
Products: -
?
gamma-butyrobetaine + 2-oxoglutarate + O2
carnitine + succinate + CO2
-
Substrates: -
Products: -
Products: -
?
L-carnitine + 2-oxoglutarate + O2
3-carboxy-N,N,N-trimethyl-2-oxopropan-1-aminium + succinate + CO2
Substrates: -
Products: -
Products: -
?
L-carnitine + 2-oxoglutarate + O2
3-carboxy-N,N,N-trimethyl-2-oxopropan-1-aminium + succinate + CO2
-
Substrates: -
Products: -
Products: -
r
[(2R)-3-carboxy-2-hydroxypropyl](trimethyl)arsanium + 2-oxoglutarate + O2
(3-carboxy-2-oxopropyl)(trimethyl)arsanium + succinate + CO2
Substrates: -
Products: -
Products: -
?
[(2R)-3-carboxy-2-hydroxypropyl](trimethyl)arsanium + 2-oxoglutarate + O2
(3-carboxy-2-oxopropyl)(trimethyl)arsanium + succinate + CO2
Substrates: -
Products: -
Products: -
?
additional information
?
-
-
Substrates: L-carnithine is an uncoupler
Products: -
Products: -
?
additional information
?
-
Substrates: no activity with trimethyllysine, acetylcholine, phosphocholine, thioacetylcholine, carbachol, and gamma-butyrobetaine analogues carboxy-N,N,N-trimethylmethanaminium, 5-carboxy-N,N,N-trimethylpentan-1-aminium, N,N,N-trimethyl-4-oxopentan-1-aminium, and 4-methoxy-N,N,N-trimethyl-4-oxobutan-1-aminium, substrate specificity, overview
Products: -
Products: -
?
additional information
?
-
-
Substrates: both enhancement of carnitine biosynthesis due to increased gamma-butyrobetaine dioxygenase activity, extra-hepatic gamma-butyrobetaine synthesis and increased hepatic carnitine import contributes to the increased hepatic carnitine levels after fasting
Products: -
Products: -
?
additional information
?
-
-
Substrates: overview on properties of substrate analogues, binding sites
Products: -
Products: -
?
additional information
?
-
-
Substrates: L-carnithine is an uncoupler
Products: -
Products: -
?
additional information
?
-
Substrates: cation-Pi interactions contribute to substrate recognition in gamma-butyrobetaine hydroxylase catalysis. BBOX contains an aromatic cage for the recognition of the positively charged trimethylammonium group of the gamma-butyrobetaine substrate. Substrate analogues (3-carboxypropyl)(trimethyl)phosphanium and (3-carboxypropyl)(trimethyl)arsanium with P and As substituting for N in the trimethylammonium group are good BBOX substrates, which follow the efficiency trend N+ >P+ >As+. An uncharged carbon analogue N-(3-hydroxypyridine-2-carbonyl)-S-[(pyridin-2-yl)methyl]-L-cysteine of gamma-butyrobetaine is not a BBOX substrate. Importance of the energetically favorable cation-Pi interactions in productive substrate recognition. Homology modeling of binding modes and structures of (3-carboxypropyl)(trimethyl)phosphanium and (3-carboxypropyl)(trimethyl)arsanium to psBBOX using an X-ray crystal structure of human BBOX in complex with ZnII, N-oxalylglycine, and gamma-butyrobetaine, PDB ID 3O2G, quantum mechanical calculations and docking simulations. Substrate specificity, overview
Products: -
Products: -
?
additional information
?
-
Substrates: no activity with trimethyllysine, acetylcholine, phosphocholine, thioacetylcholine, carbachol, and gamma-butyrobetaine analogues carboxy-N,N,N-trimethylmethanaminium, 5-carboxy-N,N,N-trimethylpentan-1-aminium, N,N,N-trimethyl-4-oxopentan-1-aminium, and 4-methoxy-N,N,N-trimethyl-4-oxobutan-1-aminium, substrate specificity, overview
Products: -
Products: -
?
additional information
?
-
Substrates: cation-Pi interactions contribute to substrate recognition in gamma-butyrobetaine hydroxylase catalysis. BBOX contains an aromatic cage for the recognition of the positively charged trimethylammonium group of the gamma-butyrobetaine substrate. Substrate analogues (3-carboxypropyl)(trimethyl)phosphanium and (3-carboxypropyl)(trimethyl)arsanium with P and As substituting for N in the trimethylammonium group are good BBOX substrates, which follow the efficiency trend N+ >P+ >As+. An uncharged carbon analogue N-(3-hydroxypyridine-2-carbonyl)-S-[(pyridin-2-yl)methyl]-L-cysteine of gamma-butyrobetaine is not a BBOX substrate. Importance of the energetically favorable cation-Pi interactions in productive substrate recognition. Homology modeling of binding modes and structures of (3-carboxypropyl)(trimethyl)phosphanium and (3-carboxypropyl)(trimethyl)arsanium to psBBOX using an X-ray crystal structure of human BBOX in complex with ZnII, N-oxalylglycine, and gamma-butyrobetaine, PDB ID 3O2G, quantum mechanical calculations and docking simulations. Substrate specificity, overview
Products: -
Products: -
?
additional information
?
-
Substrates: no activity with trimethyllysine, acetylcholine, phosphocholine, thioacetylcholine, carbachol, and gamma-butyrobetaine analogues carboxy-N,N,N-trimethylmethanaminium, 5-carboxy-N,N,N-trimethylpentan-1-aminium, N,N,N-trimethyl-4-oxopentan-1-aminium, and 4-methoxy-N,N,N-trimethyl-4-oxobutan-1-aminium, substrate specificity, overview
Products: -
Products: -
?
additional information
?
-
-
Substrates: L-carnithine is an uncoupler
Products: -
Products: -
?
additional information
?
-
-
Substrates: overview on properties of substrate analogues, binding sites
Products: -
Products: -
?
additional information
?
-
Substrates: enzyme is involved in L-carnitine (3-hydroxy-4-N-trimethylaminobutyrate) biosynthesis
Products: -
Products: -
?
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
NATURAL SUBSTRATE
NATURAL PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
LITERATURE
COMMENTARY
REVERSIBILITY
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
-
Substrates: gamma-butyrobetaine, terminal reaction in the pathway of L-carnitine biosynthesis
Products: -
Products: -
?
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
Substrates: -
Products: -
Products: -
?
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
Substrates: -
Products: -
Products: -
?
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
Substrates: -
Products: -
Products: -
?
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
Substrates: -
Products: -
Products: -
?
additional information
?
-
-
Substrates: both enhancement of carnitine biosynthesis due to increased gamma-butyrobetaine dioxygenase activity, extra-hepatic gamma-butyrobetaine synthesis and increased hepatic carnitine import contributes to the increased hepatic carnitine levels after fasting
Products: -
Products: -
?
additional information
?
-
Substrates: enzyme is involved in L-carnitine (3-hydroxy-4-N-trimethylaminobutyrate) biosynthesis
Products: -
Products: -
?
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
COFACTOR
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
additional information
-
KCl, nicotinamide, MgCl2 or catalase not required for full activity
-
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
METALS and IONS
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
Fe2+
Zn2+
Fe2+
dependent on, required for catalysis, bound to the active site
Fe2+
nonheme Fe2+-dependent oxygenase, dependent on, required for catalysis
Fe2+
nonheme Fe2+-dependent oxygenase, dependent on, required for catalysis
Fe2+
nonheme Fe2+-dependent oxygenase, dependent on, required for catalysis, bound to the active site
Zn2+
quantitative determination of Zn2+ by atomic absorption spectrometry, zinc-binding site structure in the N-terminal domain of GBBH, overview
Zn2+
dependent on, ejection of structural zinc, e.g. by Selenium and sulfur based compounds, leads to inhibition of the enzyme, an undisrupted N-terminal zinc binding domain of BBOX is required for catalysis
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INHIBITOR
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
2-(2-carboxyethyl)-1,1-dimethyl-1-(prop-2-yn-1-yl)-hydrazin-1-ium bromide
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2-(2-carboxyethyl)-1-(2-chloroethyl)-1,1-dimethylhydrazin-1-ium bromide
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3-(2,2,2-trimethylhydrazinium propionate dihydrate)
i.e. mildronate, a synthetic inhibitor of GBBH, is a non-hydroxylatable analog of gamma-butyrobetaine
3-Bromo-2-oxoglutarate
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noncompetitive inhibition, 2-oxoglutarate as variable substrate
4-([(2E)-3-carboxyprop-2-enoyl](hydroxy)amino)-N,N,N-trimethylbutan-1-aminium
i.e. RL190B
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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
Clofibrate
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activity of gamma-butyrobetaine dioxygenase in the liver is lower in pigs treated with clofibrate than in control pigs
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
p-aminomethylbenzoic acid
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1.2 mmol/kg reduces the conversion of 4-dimethylaminobutyric acid to 4-dimethylamino-3-hydroxybutyric acid from 62.6% to 46.8%
3-(1,1,1-trimethylhydrazin-1-ium-2-yl)propanoate
i.e. mildronate, a clinically used and approved drug, but weak inhibitor. Enzyme-inhibitor interactions from structure PDB ID 3O2G
4-Trimethylammoniobutanoate
substrate inhibition at concentrations above 0.2 mM
4-Trimethylammoniobutanoate
substrate inhibition at high concentrations
4-Trimethylammoniobutanoate
substrate inhibition associated with age is observed at concentrations above 0.6 mM
ascorbate
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