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Information on EC 1.14.11.1 - gamma-butyrobetaine dioxygenase and Organism(s) Mus musculus

for references in articles please use BRENDA:EC1.14.11.1
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EC Tree
IUBMB Comments
Requires Fe2+ and ascorbate.
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This record set is specific for:
Mus musculus
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Word Map
The taxonomic range for the selected organisms is: Mus musculus
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
SYNONYM
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
4-trimethylaminobutyric acid dioxygenase
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alpha-butyrobetaine hydroxylase
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-
-
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butyrobetaine hydroxylase
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-
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gamma-BBD
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gamma-butyrobetaine hydroxylase
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-
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oxygenase, gamma-butyrobetaine di-
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-
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-
REACTION TYPE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
oxidation
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-
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oxidative decarboxylation
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-
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redox reaction
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-
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reduction
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-
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PATHWAY SOURCE
PATHWAYS
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-, -
SYSTEMATIC NAME
IUBMB Comments
4-trimethylammoniobutanoate,2-oxoglutarate:oxygen oxidoreductase (3-hydroxylating)
Requires Fe2+ and ascorbate.
CAS REGISTRY NUMBER
COMMENTARY hide
9045-31-2
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SUBSTRATE
PRODUCT                       
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate) hide
LITERATURE
(Substrate)
COMMENTARY
(Product) hide
LITERATURE
(Product)
Reversibility
r=reversible
ir=irreversible
?=not specified
4-dimethylaminobutyric acid + 2-oxoglutarate + O2
4-dimethylamino-3-hydroxybutyric acid + succinate + CO2
show the reaction diagram
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-
-
-
?
additional information
?
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-
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
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-
?
NATURAL SUBSTRATE
NATURAL PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate) hide
LITERATURE
(Substrate)
COMMENTARY
(Product) hide
LITERATURE
(Product)
REVERSIBILITY
r=reversible
ir=irreversible
?=not specified
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
-
-
?
COFACTOR
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
ascorbate
-
requires the presence of ascorbate for optimal activity
METALS and IONS
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
Fe2+
-
requires the presence of Fe2+ for optimal activity
INHIBITOR
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
ZnCl2
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1 mM, complete inhibition
KM VALUE [mM]
SUBSTRATE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
0.063
2-oxoglutarate
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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
0.04
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
ORGANISM
COMMENTARY hide
LITERATURE
UNIPROT
SEQUENCE DB
SOURCE
SOURCE TISSUE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
SOURCE
UNIPROT
ENTRY NAME
ORGANISM
NO. OF AA
NO. OF TRANSM. HELICES
MOLECULAR WEIGHT[Da]
SOURCE
SEQUENCE
LOCALIZATION PREDICTION?
BODG_MOUSE
387
0
44699
Swiss-Prot
other Location (Reliability: 2)
APPLICATION
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
medicine
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both enhancement of carnitine biosynthesis due to increased ?-butyrobetaine dioxygenase activity, extra-hepatic gamma-butyrobetaine synthesis and increased hepatic carnitine import contributes to the increased hepatic carnitine levels after fasting
REF.
AUTHORS
TITLE
JOURNAL
VOL.
PAGES
YEAR
ORGANISM (UNIPROT)
PUBMED ID
SOURCE
van Vlies, N.; Wanders, R.J.; Vaz, F.M.
Measurement of carnitine biosynthesis enzyme activities by tandem mass spectrometry: differences between the mouse and the rat
Anal. Biochem.
354
132-139
2006
Mus musculus
Manually annotated by BRENDA team
van Vlies, N.; Ferdinandusse, S.; Turkenburg, M.; Wanders, R.J.; Vaz, F.M.
PPARalpha-activation results in enhanced carnitine biosynthesis and OCTN2-mediated hepatic carnitine accumulation
Biochim. Biophys. Acta
1767
1134-1142
2007
Mus musculus
Manually annotated by BRENDA team