Information on EC 1.2.1.47 - 4-trimethylammoniobutyraldehyde dehydrogenase

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

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EC NUMBER
COMMENTARY hide
1.2.1.47
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RECOMMENDED NAME
GeneOntology No.
4-trimethylammoniobutyraldehyde dehydrogenase
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REACTION
REACTION DIAGRAM
COMMENTARY hide
ORGANISM
UNIPROT
LITERATURE
4-trimethylammoniobutanal + NAD+ + H2O = 4-trimethylammoniobutanoate + NADH + 2 H+
show the reaction diagram
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-
-
-
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REACTION TYPE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
oxidation
-
-
-
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redox reaction
-
-
-
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reduction
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PATHWAY
BRENDA Link
KEGG Link
MetaCyc Link
L-carnitine biosynthesis
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carnitine metabolism
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Lysine degradation
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SYSTEMATIC NAME
IUBMB Comments
4-trimethylammoniobutanal:NAD+ 1-oxidoreductase
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CAS REGISTRY NUMBER
COMMENTARY hide
73361-01-0
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ORGANISM
COMMENTARY hide
LITERATURE
UNIPROT
SEQUENCE DB
SOURCE
-
-
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Manually annotated by BRENDA team
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UniProt
Manually annotated by BRENDA team
Wistar strain
UniProt
Manually annotated by BRENDA team
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GENERAL INFORMATION
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
malfunction
-
enzyme overexpression causes the Kawasaki disease (KD), an acute vasculitis that preferentially affects coronary arteries. The disease is still the leading cause of acquired heart disease in children. Patients suffering Kawasaki disease show increased TMABADH enzyme protein levels compared to controls
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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-dimethylaminobutyraldehyde + NAD+
4-dimethylaminobutanoate + NADH
show the reaction diagram
4-dimethylaminobutyraldehyde + NAD+ + H2O
? + NADH + H+
show the reaction diagram
4-N-trimethylaminobutyraldehyde + NAD+
4-N-trimethylaminobutanoate + NADH
show the reaction diagram
4-N-trimethylaminobutyraldehyde + NAD+ + H2O
gamma-butyrobetaine + NADH + H+
show the reaction diagram
4-trimethylaminobutyraldehyde + NAD+
4-trimethylaminobutanoate + NADH
show the reaction diagram
4-trimethylaminobutyraldehyde + NAD+
4-trimethylaminobutyraldehyde + NADH
show the reaction diagram
4-trimethylaminobutyraldehyde + NAD+
4-trimethylaminobutyrate + NADH
show the reaction diagram
4-trimethylammoniobutanal + NAD+ + H2O
4-trimethylammoniobutanoate + NADH + 2 H+
show the reaction diagram
acetaldehyde + NAD+ + H2O
acetate + NADH + H+
show the reaction diagram
betaine aldehyde + NAD+
betaine + NADH
show the reaction diagram
-
-
?
butyraldehyde + NAD+
butyrate + NADH
show the reaction diagram
gamma-aminobutyraldehyde + NAD+
gamma-aminobutyrate + NADH
show the reaction diagram
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-
?
gamma-aminobutyraldehyde + NAD+ + H2O
4-aminobutanoate + NADH + H+
show the reaction diagram
-
-
-
?
heptanal + NAD+
heptanoate + NADH
show the reaction diagram
-
-
?
hexadecanal + NAD+
hexadecanoate + NADH
show the reaction diagram
-
-
?
hexanal + NAD+ + H2O
hexanoate + NADH + H+
show the reaction diagram
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-
?
octanal + NAD+ + H2O
octanoate + NADH + H+
show the reaction diagram
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-
?
pentanal + NAD+
pentanoate + NADH
show the reaction diagram
-
-
?
propionaldehyde + NAD+
propionate + NADH
show the reaction diagram
trimethylaminobutyraldehyde + NAD+ + H2O
gamma-butyrobetaine + NADH + 2 H+
show the reaction diagram
enzyme is involved in L-carnitine (3-hydroxy-4-N-trimethylaminobutyrate) biosynthesis
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-
?
additional information
?
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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-N-trimethylaminobutyraldehyde + NAD+ + H2O
gamma-butyrobetaine + NADH + H+
show the reaction diagram
4-trimethylaminobutyraldehyde + NAD+
4-trimethylaminobutanoate + NADH
show the reaction diagram
4-trimethylaminobutyraldehyde + NAD+
4-trimethylaminobutyraldehyde + NADH
show the reaction diagram
4-trimethylammoniobutanal + NAD+ + H2O
4-trimethylammoniobutanoate + NADH + 2 H+
show the reaction diagram
trimethylaminobutyraldehyde + NAD+ + H2O
gamma-butyrobetaine + NADH + 2 H+
show the reaction diagram
Q9JLJ3
enzyme is involved in L-carnitine (3-hydroxy-4-N-trimethylaminobutyrate) biosynthesis
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-
?
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COFACTOR
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
NADP+
can also be used as oxidant
additional information
-
does not react with NADP+
-
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METALS and IONS
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
additional information
Rb+, Na+, Mg2+, and Ca2+ have no effect on the enzyme activity
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INHIBITORS
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
1,10-phenanthroline
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1 mM causes 5% inhibition at 30°C
3-methylbutyraldehyde
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1 mM causes 57% inhibition at 30°C
4-chloromercuribenzoate
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4-N-trimethylaminobutyraldehyde
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the substrate inhibits at 0.24 mM
5,5'-dithiobis-(2-nitrobenzoic acid)
acetaldehyde
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1 mM causes 18% inhibition at 30°C
Betaine aldehyde
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1 mM causes 41% inhibition at 30°C
diethylmethylamine
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at 30°C, 5 mM causes 53% inhibition, does not inhibit at 1 mM
dimethylethylamine
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5 mM causes 9% inhibition at 30°C
Disulfiram
ethylmethylamine
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1 mM causes 20% inhibition at 30°C
glyceraldehyde
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1 mM causes 7% inhibition at 30°C
iodoacetoamide
KCl
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1 mM causes 5% inhibition at 30°C
p-chloromercuribenzoate
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0.1 mM causes 88% inhibition at 30°C
phenylacetaldehyde
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0.1 mM causes 54% inhibition at 30°C
Semicarbazide
triethylamine
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5 mM causes 42% inhibition at 30°C
trimethylacetaldehyde
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1 mM causes 80% inhibition at 30°C
additional information
-
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KM VALUE [mM]
SUBSTRATE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
0.024
4-Aminobutyraldehyde
-
0.051 - 0.62
4-dimethylaminobutyraldehyde
0.0074 - 0.31
4-N-trimethylaminobutyraldehyde
0.0014 - 0.00417
4-trimethylaminobutyraldehyde
0.102
acetaldehyde
-
0.123
Betaine aldehyde
-
0.013
Butyraldehyde
-
0.007
Heptanal
-
0.0088
hexadecanal
-
0.009
Hexanal
-
0.028 - 1.16
NAD+
0.006
octanal
-
0.01
pentanal
-
0.016
propionaldehyde
-
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SPECIFIC ACTIVITY [µmol/min/mg]
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
0.0000077
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muscle
0.000179
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heart
0.000298
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brain
0.001445
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kidney
0.001698
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testis
0.002138
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liver
0.21
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activity on propionaldehyde
0.772
purified enzyme
5.1
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activity on 4-trimethylaminobutyraldehyde
15.4
recombinant enzyme, after 19.5fold purification, at pH 9.5 and 30°C
additional information
expression of TMABA-DH is ontogenically regulated, renal levels of TMABA-DH are quantified by real-time PCR assays in foetuses, newborn, suckling, weaning and 2-month-old rats, foetuses express TMABA-DH mRNA and this expression increases significantly with maturation, comparisons between cortex and medulla reveals that, except for foetuses and newborn rats, TMABA-DHmRNA levels are significantly higher in the medulla than in the cortex; levels of liver mRNA TMABA-DH are approximately 2times greater than the maximal values measured in kidneys
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pH OPTIMUM
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
9
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TMABA-DH activity assay
9.5 - 9.8
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pH RANGE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
6.2 - 10.5
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pH 6.2: about 30% of activity maximum, pH 10.5: about 15% of activity maximum
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TEMPERATURE OPTIMUM
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
25
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TMABA-DH activity assay
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pI VALUE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
4.5
isoelectric focusing
5.5
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isoelectric focusing
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SOURCE TISSUE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
SOURCE
-
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Manually annotated by BRENDA team
additional information
alpha-Smooth muscle actin, a marker for endothelial cells, does not colocalize with TMABA-DH signals. The rat enzyme can be detected wit ant-human TMABA-DH antibodies in rat hearts
Manually annotated by BRENDA team
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LOCALIZATION
ORGANISM
UNIPROT
COMMENTARY hide
GeneOntology No.
LITERATURE
SOURCE
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MOLECULAR WEIGHT
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
52000
3 * 52000, SDS-PAGE
150000
gel filtration
160000
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SUBUNITS
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
?
x * 55000, SDS-PAGE
homotrimer
trimer
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pH STABILITY
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
8 - 10
after pre-incubation for 15 min at 30°C, the enzyme is stable between pH 8.0 and 9.0 but is inactivated completely at pH 10.0
726537
8 - 9
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the enzyme is completely inactivated at pH 9.6
691376
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TEMPERATURE STABILITY
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
4
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0.1% 2-mercaptoethanol, 20% glycerol, fairly stable, but storage for several weeks results in loss of most of the activity
25 - 30
the enzyme is stable up to 25°C while incubation for 30 min at 30°C and above leads to rapid inactivation
30
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the enzyme is rapidly inactivated at temperatures above 35°C
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STORAGE STABILITY
ORGANISM
UNIPROT
LITERATURE
-80°C, 0.1% 2-mercaptoethanol, 20% glycerol, several months
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-80°C, 1 mM dithiothreitol, glycerol, several months
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Purification/COMMENTARY
ORGANISM
UNIPROT
LITERATURE
14.2fold to apparent homogeneity, by hydrophobic chromatography and affinity chromatography
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ammonium sulfate precipitation, phenyl-Toyopearl column chromatography, and 5'-AMP Sepharose column chromatography
recombinant FLAG-tagged enzyme TMABA-DH from HEK-293T cells by immunoaffinity chromatography
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using column chromatography on blue dextran-Sepharose and 5'-AMP-Sepharose
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using column chromatography on SP-Sepharose, Red Sepharose CL-6B and Hydroxylapatite
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Cloned/COMMENTARY
ORGANISM
UNIPROT
LITERATURE
expressed in Escherichia coli DH5alpha and BL21 (DE3) cells
expression in Escherichia coli
recombinant expression of FLAG-tagged enzyme TMABA-DH in HEK-293T cells
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EXPRESSION
ORGANISM
UNIPROT
LITERATURE
detection of mRNA levels in foetuses, increase during postnatal life, expression is ontogenically regulated
the enzyme gene is activated by peroxisome proliferator-activated receptor alpha directly and by WY-14,643 in the liver of wild type mice
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APPLICATION
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
diagnostics
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detection of anti-TMABA-DH autoantibody is a potential strategy for a diagnosis of Kawasaki disease, usefulness of the anti-TMABA-DH antibody as a diagnostic marker
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LINKS TO OTHER DATABASES (specific for EC-Number 1.2.1.47)
ExplorEnz
ExPASy
KEGG
MetaCyc
SABIO-RK
NCBI: PubMed, Protein, Nucleotide, Structure, Genome, OMIM
IUBMB Enzyme Nomenclature
PROSITE Database of protein families and domains
InterPro (database of protein families, domains and functional sites)