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EC Tree
The taxonomic range for the selected organisms is: Rattus norvegicus The expected taxonomic range for this enzyme is: Eukaryota, Bacteria
Synonyms
gamma-butyrobetaine hydroxylase, bbox1, butyrobetaine hydroxylase, gamma-butyrobetaine dioxygenase, gamma-butyrobetaine hydroxylase 1, alpha-butyrobetaine hydroxylase, bu hydroxylase, gbb hydroxylase,
more
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gamma-butyrobetaine hydroxylase
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alpha-butyrobetaine hydroxylase
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butyrobetaine hydroxylase
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gamma butyrobetaine hydroxylase
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gamma-buryrobetaine dioxygenase
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gamma-butyrobetaine hydroxylase
oxygenase, gamma-butyrobetaine di-
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gamma-butyrobetaine hydroxylase
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gamma-butyrobetaine hydroxylase
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oxidative decarboxylation
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4-trimethylammoniobutanoate,2-oxoglutarate:oxygen oxidoreductase (3-hydroxylating)
Requires Fe2+ and ascorbate.
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3-trimethylaminopropionic acid + 2-oxoglutarate + O2
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20% of the hydroxylation rate of gamma-butyrobetaine
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?
4-dimethylaminobutyric acid + 2-oxoglutarate + O2
4-dimethylamino-3-hydroxybutyric acid + succinate + CO2
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
D-carnitine + 2-oxoglutarate + O2
3-carboxy-N,N,N-trimethyl-2-oxopropan-1-aminium + succinate + CO2
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r
L-carnitine + 2-oxoglutarate + O2
3-carboxy-N,N,N-trimethyl-2-oxopropan-1-aminium + succinate + CO2
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r
additional information
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enzyme is involved in L-carnitine (3-hydroxy-4-N-trimethylaminobutyrate) biosynthesis
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4-dimethylaminobutyric acid + 2-oxoglutarate + O2
4-dimethylamino-3-hydroxybutyric acid + succinate + CO2
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4-dimethylaminobutyric acid + 2-oxoglutarate + O2
4-dimethylamino-3-hydroxybutyric acid + succinate + CO2
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poor substrate
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?
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
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4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
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?
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
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?
4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
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4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
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4-trimethylammoniobutanoate + 2-oxoglutarate + O2
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2
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i.e. gamma-butyrobetaine
i.e. L-carnitine
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additional information
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enzyme is involved in L-carnitine (3-hydroxy-4-N-trimethylaminobutyrate) biosynthesis
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2,6-dichlorophenolindophenol
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additional information
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KCl, nicotinamide, MgCl2 or catalase not required for full activity
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ascorbate
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ascorbate
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activation, keeps Fe2+ in the reduced state
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2-oxoglutarate
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at concentrations above 1 mM
3-(2,2,2-trimethylhydrazinium)propionate
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complete inhibition at 0.05 mM
3-(2,2,2-trimethylhydrazinium)propionate dihydrate
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3-Bromo-2-oxoglutarate
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noncompetitive inhibition, 2-oxoglutarate as variable substrate
3-glutathione-2-oxoglutarate
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noncompetitive to 2-oxoglutarate
3-trimethylaminopropyl-1-sulfonate
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FMN
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in high concentrations
gamma-butyrobetaine
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at concentrations above 0.2 mM
iodoacetate
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less effective than p-chloromercuriphenylsulfonate
Iodosobenzoate
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less effective than p-chloromercuriphenylsulfonate
N-ethylmaleimide
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less effective than p-chloromercuriphenylsulfonate
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%
p-chloromercuribenzoate
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inactivates enzyme completely at 0.1 mM
p-chloromercuriphenylsulfonate
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inactivates enzyme completely at 0.1 mM
structure analogues of gamma-butyrobetaine and 2-oxoglutarate
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Succinic semialdehyde
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2-amino-6,7-dimethyl-4-hydroxy-5,6,7,8-tetrahydropteridine
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Cs+
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increase of activity
GSH/GSH-peroxidase
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increase of activity, more efficient in assay and during preincubation than catalase, protects the enzyme from increasing phosphate concentrations
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isoascorbate
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increase of activity
K+
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efficient coupling of decarboxylation and hydroxylation, stimulation
Microsomal preparation
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increase of activity
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NADPH
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NADPH-regenerating system, increase of activity, no absolute requirement
NH4+
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increase of activity
nicotinamide
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increase of activity
Rb+
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increase of activity
catalase
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catalase
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increase of activity
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catalase
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protection from inactivation
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0.125
2-oxo-glutarate
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0.029 - 0.08
gamma-butyrobetaine
additional information
additional information
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0.029
gamma-butyrobetaine
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0.08
gamma-butyrobetaine
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additional information
BBH mRNA is detected during the suckling period onwards and its abundance is not changed significantly by maturation, expression of BBH mRNA is undetectable in foetuses and newborn rats, it is measurable in suckling rats, no significant differences are observed between cortical and medullar BBH mRNA levels
additional information
levels of liver mRNA BBH are approx. 650times and 2times greater than the maximal values measured in kidneys
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6 - 7.5
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half-maximal activity at pH 6.0 and 7.5
6 - 8.4
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half-maximal activity at pH 6.0 and 8.4, partially purified preparation
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foetuses, newborn, suckling, weaning and adult rats
UniProt
brenda
male Wister rat
UniProt
brenda
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brenda
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brenda
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brenda
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brenda
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brenda
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brenda
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brenda
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the enzyme is expressed but without activity in renal tissue
brenda
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439283 , 439285 , 439290 , 439291 , 439297 , 439299 , 439300 , 439301 , 439302 , 439303 , 675561 , 675785 brenda
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brenda
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brenda
additional information
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no activity found: muscle, kidney
brenda
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brenda
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brenda
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physiological function
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biosynthesis of carnitine, important role in beta-oxidation of fatty acid
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BODG_RAT
387
0
44546
Swiss-Prot
other Location (Reliability: 2 )
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43000
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2 * 43000, SDS-PAGE
44000
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x * 44000, SDS-PAGE
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dimer
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2 * 43000, SDS-PAGE
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60
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complete inactivation after 15 min
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O2, irreversible inactivation during preincubation
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439297
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4°C, 50 mM sodium phosphate pH 7.4, 20 mM KCl, 10 mM DTT, 200g/l glycerol are best conservation conditions
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addition of EDTA causes total loss of activity
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expression in HEK293 cells
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detection of mRNA levels during suckling period onwards, expression is ontogenically regulated
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medicine
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effects of hyperthyroidism and hypothyroidism on enzyme activity
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Lindstedt, G.; Lindstedt, S.
Cofactor requirements of gamma-butyrobetaine hydroxylase from rat liver
J. Biol. Chem.
245
4178-4186
1970
Rattus norvegicus
brenda
Carter, A.L.; Stratman, F.W.
A rapid and sensitive assay of gamma-butyrobetaine hydroxylase
FEBS Lett.
111
112-114
1980
Rattus norvegicus
brenda
Punekar, N.S.; Wehbie, R.S.; Lardy, H.A.
gamma-Butyrobetaine hydroxylase and the protective role of glutathione peroxidase
J. Biol. Chem.
262
6720-6724
1987
Rattus norvegicus
brenda
Wehbie, R.S.; Punekar, N.S.; Lardy, H.A.
Rat liver gamma-butyrobetaine hydroxylase catalyzed reaction: influence of potassium, substrates, and substrate analogues on hydroxylation and decarboxylation
Biochemistry
27
2222-2228
1988
Rattus norvegicus
brenda
Lindstedt, G.
Hydroxylation of gamma-butyrobetaine to carnitine in rat liver
Biochemistry
6
1271-1282
1967
Rattus norvegicus
brenda
Paul, H.S.; Sekas, G.; Adibi, S.A.
Carnitine biosynthesis in hepatic peroxisomes. Demonstration of gamma-butyrobetaine hydroxylase activity
Eur. J. Biochem.
203
599-605
1992
Rattus norvegicus
brenda
Vaz, F.M.; van Gool, S.; Ofman, R.; Ijlst, L.; Wanders, R.J.
Carnitine biosynthesis. Purification of gamma-butyrobetaine hydroxylase from rat liver
Adv. Exp. Med. Biol.
466
117-124
1999
Rattus norvegicus
brenda
Galland, S.; Le Borgne, F.; Guyonnet, D.; Clouet, P.; Demarquoy, J.
Purification and characterization of the rat liver gamma-butyrobetaine hydroxylase
Mol. Cell. Biochem.
178
163-168
1998
Rattus norvegicus
brenda
Galland, S.; Georges, B.; Le Borgne, F.; Conductier, G.; Dias, J.V.; Demarquoy, J.
Thyroid hormone controls carnitine status through modifications of g-butyrobetaine hydroxylase activity and gene expression
Cell. Mol. Life Sci.
59
540-545
2002
Rattus norvegicus
brenda
Galland, S.; Le Borgne, F.; Bouchard, F.; Georges, B.; Clouet, P.; Grand-Jean, F.; Demarquoy, J.
Molecular cloning and characterization of the cDNA encoding the rat liver gamma-butyrobetaine hydroxylase
Biochim. Biophys. Acta
1441
85-92
1999
Rattus norvegicus
brenda
Davis, A.T.; Monroe, T.J.
Carnitine deficiency and supplementation do not affect the gene expression of carnitine biosynthetic enzymes in rats
J. Nutr.
135
761-764
2005
Rattus norvegicus
brenda
Debreceni, B.; Farkas, V.; Fischer, G.M.; Sandor, A.
Effect of aromatic ring-containing drugs on carnitine biosynthesis in rats with special regard to p-aminomethylbenzoic acid
Metab. Clin. Exp.
54
1582-1586
2005
Rattus norvegicus
brenda
Buermans, H.P.; Redout, E.M.; Schiel, A.E.; Musters, R.J.; Zuidwijk, M.; Eijk, P.P.; van Hardeveld, C.; Kasanmoentalib, S.; Visser, F.C.; Ylstra, B.; Simonides, W.S.
Microarray analysis reveals pivotal divergent mRNA expression profiles early in the development of either compensated ventricular hypertrophy or heart failure
Physiol. Genomics
21
314-323
2005
Rattus norvegicus
brenda
Fujita, M.; Nakanishi, T.; Shibue, Y.; Kobayashi, D.; Moseley, R.H.; Shirasaka, Y.; Tamai, I.
Hepatic uptake of gamma-butyrobetaine, a precursor of carnitine biosynthesis, in rats
Am. J. Physiol. Gastrointest. Liver Physiol.
297
G681-G686
2009
Rattus norvegicus
brenda
Garcia-Delgado, M.; Peral, M.J.; Duran, J.M.; Garcia-Miranda, P.; Calonge, M.L.; Ilundain, A.A.
Ontogeny of Na(+)/L-carnitine transporter and of gamma-trimethylaminobutyraldehyde dehydrogenase and gamma-butyrobetaine hydroxylase genes expression in rat kidney
Mech. Ageing Dev.
130
227-233
2009
Rattus norvegicus (Q9QZU7)
brenda