1.14.11.1: gamma-butyrobetaine dioxygenase
This is an abbreviated version!
For detailed information about gamma-butyrobetaine dioxygenase, go to the full flat file.
Word Map on EC 1.14.11.1
-
1.14.11.1
-
cardioprotective
-
carnitine-dependent
-
3-2,2,2-trimethylhydrazinium
-
mildronate
-
trimethyllysine
-
mid1
-
alpha4
-
medicine
-
d-carnitine
-
dihydrate
-
drug development
-
molecular biology
- 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
Synonyms
4-trimethylaminobutyric acid dioxygenase, alpha-butyrobetaine hydroxylase, BBD, BBH, BBOX, BBOX1, Bu hydroxylase, butyrobetaine hydroxylase, gamma butyrobetaine hydroxylase, gamma-BBD, gamma-BBH, gamma-buryrobetaine dioxygenase, gamma-butyrobetaine dioxygenase, gamma-butyrobetaine hydroxylase, gamma-butyrobetaine hydroxylase 1, GBB hydroxylase, GBBH, hBBOX, oxygenase, gamma-butyrobetaine di-, psBBOX
ECTree
1.14.11.1 gamma-butyrobetaine dioxygenaseAdvanced search results
results (
results (Substrates Products
Substrates Products on EC 1.14.11.1 - gamma-butyrobetaine dioxygenase
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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
(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
?
-
weak substrate, below 5% turnover after 3 h
-
-
?
(3S)-3-fluoro-4-(trimethylammonio)butanoate + 2-oxoglutarate + O2
?
-
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
(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
-
-
-
?
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
?
-
20% of the hydroxylation rate of gamma-butyrobetaine
-
-
?
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
-
-
-
?
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
?
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
[(2R)-3-carboxy-2-hydroxypropyl](trimethyl)phosphanium + 2-oxoglutarate + O2
(3-carboxy-2-oxopropyl)(trimethyl)phosphanium + succinate + CO2
-
-
-
?
[(2S)-3-carboxy-2-hydroxypropyl](trimethyl)arsanium + 2-oxoglutarate + O2
(3-carboxy-2-oxopropyl)(trimethyl)arsanium + succinate + CO2
-
-
-
?
[(2S)-3-carboxy-2-hydroxypropyl](trimethyl)phosphanium + 2-oxoglutarate + O2
(3-carboxy-2-oxopropyl)(trimethyl)phosphanium + succinate + CO2
-
-
-
?
(3-carboxypropyl)(trimethyl)arsanium + 2-oxoglutarate + O2
[(2R)-3-carboxy-2-hydroxypropyl](trimethyl)arsanium + succinate + CO2
-
-
-
?
(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
-
-
-
?
(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
3-carboxy-N,N,N-trimethyl-2-oxopropan-1-aminium + succinate + formylfluoride
-
-
-
?
(3R)-3-fluoro-4-(trimethylammonio)butanoate + 2-oxoglutarate + O2
3-carboxy-N,N,N-trimethyl-2-oxopropan-1-aminium + succinate + formylfluoride
-
-
-
?
(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
-
-
-
?
(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)-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
-
-
-
?
2-carboxy-N,N,N-trimethylethan-1-aminium + 2-oxoglutarate + O2
4-carboxy-3-hydroxy-N,N,N-trimethylbutan-1-aminium + succinate + CO2
-
-
-
?
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-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
-
-
-
?
4-dimethylaminobutyric acid + 2-oxoglutarate + O2
4-dimethylamino-3-hydroxybutyric acid + succinate + CO2
-
-
-
-
?
4-dimethylaminobutyric acid + 2-oxoglutarate + O2
4-dimethylamino-3-hydroxybutyric acid + succinate + CO2
-
-
-
-
?
4-dimethylaminobutyric acid + 2-oxoglutarate + O2
4-dimethylamino-3-hydroxybutyric acid + succinate + CO2
-
poor substrate
-
?
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
-
-
-
?
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
-
-
-
?
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
-
gamma-butyrobetaine, terminal reaction in the pathway of L-carnitine biosynthesis
-
-
?
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
-
-
-
?
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
-
-
-
?
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
-
-
-
?
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
-
-
-
?
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
catalytic domain and active site structure, overview
-
-
?
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
-
-
-
?
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
-
-
-
?
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
-
-
-
?
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
-
-
-
?
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
-
-
-
?
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
-
-
-
?
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
-
-
-
?
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
-
-
-
?
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
-
-
-
?
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
-
-
-
?
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
-
-
-
?
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
-
-
-
?
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
-
-
-
?
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
-
-
-
?
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
-
-
-
?
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
-
-
-
?
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
-
-
-
?
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
-
-
-
?
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
-
-
-
-
?
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
-
i.e. gamma-butyrobetaine
i.e. L-carnitine
?
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
-
-
-
?
5,5-dimethylhexanoate + 2-oxoglutarate + O2
?
-
poor substrate, 20% of the decarboxylation events lead to hydroxylation
-
-
?
5,5-dimethylhexanoate + 2-oxoglutarate + O2
?
-
poor substrate, 20% of the decarboxylation events lead to hydroxylation
-
-
?
D-carnitine + 2-oxoglutarate + O2
3-carboxy-N,N,N-trimethyl-2-oxopropan-1-aminium + succinate + CO2
-
-
-
?
D-carnitine + 2-oxoglutarate + O2
3-carboxy-N,N,N-trimethyl-2-oxopropan-1-aminium + succinate + CO2
-
-
-
?
D-carnitine + 2-oxoglutarate + O2
3-carboxy-N,N,N-trimethyl-2-oxopropan-1-aminium + succinate + CO2
-
-
-
-
r
L-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
-
-
-
-
r
[(2R)-3-carboxy-2-hydroxypropyl](trimethyl)arsanium + 2-oxoglutarate + O2
(3-carboxy-2-oxopropyl)(trimethyl)arsanium + succinate + CO2
-
-
-
?
[(2R)-3-carboxy-2-hydroxypropyl](trimethyl)arsanium + 2-oxoglutarate + O2
(3-carboxy-2-oxopropyl)(trimethyl)arsanium + succinate + CO2
-
-
-
?
additional information
?
-
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
-
-
?
additional information
?
-
-
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
-
-
?
additional information
?
-
-
overview on properties of substrate analogues, binding sites
-
-
?
additional information
?
-
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
-
-
?
additional information
?
-
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
-
-
?
additional information
?
-
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
-
-
?
additional information
?
-
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
-
-
?
additional information
?
-
-
overview on properties of substrate analogues, binding sites
-
-
?
additional information
?
-
enzyme is involved in L-carnitine (3-hydroxy-4-N-trimethylaminobutyrate) biosynthesis
-
-
?
