HOME
login
history
all enzymes
BRENDA home
History
Information on EC 1.2.1.47 - 4-trimethylammoniobutyraldehyde dehydrogenase
for references in articles please use BRENDA:EC1.2.1.47Please wait a moment until all data is loaded. This message will disappear when all data is loaded.
EC Tree
1.2.1.47 4-trimethylammoniobutyraldehyde dehydrogenaseSpecify your search results
Word Map
- 1.2.1.47
- gamma-butyrobetaine
-
proliferator-activated
-
dioxygenase
- peroxisome
- l-carnitine
-
polyunsaturated
-
1.2.1.8
-
badhs
-
aldh4a1
-
suckling
-
gill
- betaine
- acetaldehyde
- trimethyllysine
The expected taxonomic range for this enzyme is: Eukaryota, Bacteria
Reaction Schemes
Synonyms
4-N-trimethylaminobutyraldehyde dehydrogenase, 4-N-trimethylaminobutyraldehyde dehydrogenase II, 4-trimethylaminobutyraldehyde dehydrogenase, 4-trimethylammoniobutyraldehyde dehydrogenase, Aldh9a1, carnitine biosynthesis enzyme, dehydrogenase, trimethylaminobutyraldehyde, gamma-trimethylaminobutyraldehyde dehydrogenase, TMABA dehydrogenase, TMABA-DH, 
more
topPlease wait a moment until the data is sorted. This message will disappear when the data is sorted.
SYNONYM 
ORGANISM
UNIPROT
COMMENTARY 
LITERATURE
4-N-trimethylaminobutyraldehyde dehydrogenase
4-N-trimethylaminobutyraldehyde dehydrogenase II
4-trimethylaminobutyraldehyde dehydrogenase
4-trimethylammoniobutyraldehyde dehydrogenase
Aldh9a1
dehydrogenase, trimethylaminobutyraldehyde
-
-
-
-
TMABA dehydrogenase
TMABA-DH
TMABaldehyde-DH II
4-N-trimethylaminobutyraldehyde dehydrogenase
-
-
-
-
4-trimethylaminobutyraldehyde dehydrogenase
-
-
-
-
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
REACTION
REACTION DIAGRAM
COMMENTARY
ORGANISM
UNIPROT
LITERATURE
4-trimethylammoniobutanal + NAD+ + H2O = 4-trimethylammoniobutanoate + NADH + 2 H+
-
-
-
-
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
REACTION TYPE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
oxidation
-
-
-
-
redox reaction
-
-
-
-
reduction
reduction
-
-
-
-
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
PATHWAY SOURCE
PATHWAYS
BRENDA
KEGG
MetaCyc
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
SYSTEMATIC NAME
IUBMB Comments
4-trimethylammoniobutanal:NAD+ 1-oxidoreductase
-
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
CAS REGISTRY NUMBER
COMMENTARY
73361-01-0
-
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
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
gamma-aminobutyraldehyde + NAD+
gamma-aminobutyrate + NADH
-
-
?
gamma-aminobutyraldehyde + NAD+ + H2O
4-aminobutanoate + NADH + H+
-
-
-
?
trimethylaminobutyraldehyde + NAD+ + H2O
gamma-butyrobetaine + NADH + 2 H+
enzyme is involved in L-carnitine (3-hydroxy-4-N-trimethylaminobutyrate) biosynthesis
-
-
?
4-dimethylaminobutyraldehyde + NAD+
4-dimethylaminobutanoate + NADH
-
-
-
-
?
4-dimethylaminobutyraldehyde + NAD+
4-dimethylaminobutanoate + NADH
-
-
-
-
?
4-N-trimethylaminobutyraldehyde + NAD+
4-N-trimethylaminobutanoate + NADH
-
-
-
-
?
4-N-trimethylaminobutyraldehyde + NAD+
4-N-trimethylaminobutanoate + NADH
-
-
-
-
?
4-N-trimethylaminobutyraldehyde + NAD+ + H2O
gamma-butyrobetaine + NADH + H+
-
-
-
-
?
4-N-trimethylaminobutyraldehyde + NAD+ + H2O
gamma-butyrobetaine + NADH + H+
-
-
-
?
4-N-trimethylaminobutyraldehyde + NAD+ + H2O
gamma-butyrobetaine + NADH + H+
-
-
-
?
4-trimethylaminobutyraldehyde + NAD+
4-trimethylaminobutanoate + NADH
-
-
-
-
?
4-trimethylaminobutyraldehyde + NAD+
4-trimethylaminobutyraldehyde + NADH
-
function in carnitine biosynthesis
-
?
4-trimethylaminobutyraldehyde + NAD+
4-trimethylaminobutyraldehyde + NADH
-
-
?
4-trimethylaminobutyraldehyde + NAD+
4-trimethylaminobutyrate + NADH
-
-
-
?
4-trimethylaminobutyraldehyde + NAD+
4-trimethylaminobutyrate + NADH
-
-
?
4-trimethylammoniobutanal + NAD+ + H2O
4-trimethylammoniobutanoate + NADH + 2 H+
-
-
-
-
?
4-trimethylammoniobutanal + NAD+ + H2O
4-trimethylammoniobutanoate + NADH + 2 H+
-
-
-
-
?
4-trimethylammoniobutanal + NAD+ + H2O
4-trimethylammoniobutanoate + NADH + 2 H+
-
-
-
?
additional information
?
-
-
does not degradate 4-trimethylaminobutanol, betaine aldehyde, 4-aminobutyraldehyde, acetaldehyde, propionaldehyde, butyraldehyde, trimethylacetaldehyde, and 3-methylbutyraldehyde
-
-
-
additional information
?
-
no activity with NADP+, trimethylaminobutanol, 3-N-trimethylaminopropionaldehyde, betaine aldehyde, 4-aminobutyraldehyde, and other aliphatic aldehydes such as acetaldehyde, propionaldehyde, butyraldehyde, pivaleraldehyde, isovaleraldehyde, butyraldehyde, trimethylacetaldehyde, and 3-methylbutyraldehyde
-
-
-
additional information
?
-
-
does not degradate 4-trimethylaminobutanol, betaine aldehyde, 4-aminobutyraldehyde, acetaldehyde, propionaldehyde, butyraldehyde, trimethylacetaldehyde, and 3-methylbutyraldehyde
-
-
-
additional information
?
-
no activity with NADP+, trimethylaminobutanol, 3-N-trimethylaminopropionaldehyde, betaine aldehyde, 4-aminobutyraldehyde, and other aliphatic aldehydes such as acetaldehyde, propionaldehyde, butyraldehyde, pivaleraldehyde, isovaleraldehyde, butyraldehyde, trimethylacetaldehyde, and 3-methylbutyraldehyde
-
-
-
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
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
trimethylaminobutyraldehyde + NAD+ + H2O
gamma-butyrobetaine + NADH + 2 H+
Q9JLJ3
enzyme is involved in L-carnitine (3-hydroxy-4-N-trimethylaminobutyrate) biosynthesis
-
-
?
4-N-trimethylaminobutyraldehyde + NAD+ + H2O
gamma-butyrobetaine + NADH + H+
-
-
-
-
?
4-N-trimethylaminobutyraldehyde + NAD+ + H2O
gamma-butyrobetaine + NADH + H+
N0DT23
-
-
-
?
4-N-trimethylaminobutyraldehyde + NAD+ + H2O
gamma-butyrobetaine + NADH + H+
N0DT23
-
-
-
?
4-trimethylaminobutyraldehyde + NAD+
4-trimethylaminobutanoate + NADH
-
-
-
-
?
4-trimethylaminobutyraldehyde + NAD+
4-trimethylaminobutyraldehyde + NADH
-
function in carnitine biosynthesis
-
?
4-trimethylaminobutyraldehyde + NAD+
4-trimethylaminobutyraldehyde + NADH
Q9JLJ3
-
-
?
4-trimethylammoniobutanal + NAD+ + H2O
4-trimethylammoniobutanoate + NADH + 2 H+
-
-
-
-
?
4-trimethylammoniobutanal + NAD+ + H2O
4-trimethylammoniobutanoate + NADH + 2 H+
-
-
-
-
?
4-trimethylammoniobutanal + NAD+ + H2O
4-trimethylammoniobutanoate + NADH + 2 H+
Q9JLJ3
-
-
-
?
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
COFACTOR
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
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
additional information
Rb+, Na+, Mg2+, and Ca2+ have no effect on the enzyme activity
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
INHIBITOR
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
diethylmethylamine
-
at 30°C, 5 mM causes 53% inhibition, does not inhibit at 1 mM
additional information
-
at 30°C, not inhibited by Rb+, Na+, Mg2+, Ca2+, potassium fluoride, 1 mM sodium azide, 2,2'-dipyridyl, EDTA, 1 mM trimethylamine and 1 mM tetramethylammonium chloride
-
additional information
potassium fluoride, sodium azide, 2,2'-dipyridyl, and EDTA have no effect on the enzyme activity
-
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
DISEASE
TITLE OF PUBLICATION
LINK TO PUBMED
Mucocutaneous Lymph Node Syndrome
Identification of 4-Trimethylaminobutyraldehyde Dehydrogenase (TMABA-DH) as a Candidate Serum Autoantibody Target for Kawasaki Disease.
Polycystic Ovary Syndrome
A nontargeted proteomic study of the influence of androgen excess on human visceral and subcutaneous adipose tissue proteomes.
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
KM VALUE [mM]
SUBSTRATE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
SPECIFIC ACTIVITY [µmol/min/mg]
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
0.8
12.4
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
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
pH OPTIMUM
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
pH RANGE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
6.2 - 10.5
-
pH 6.2: about 30% of activity maximum, pH 10.5: about 15% of activity maximum
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
TEMPERATURE OPTIMUM
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
40
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
pI VALUE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
ORGANISM
COMMENTARY
LITERATURE
UNIPROT
SEQUENCE DB
SOURCE
foetuses, newborn, suckling, weaning and adult rats; male Wister rat
UniProt
brenda
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
SOURCE TISSUE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
SOURCE
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
brenda
enzyme TMABA-DH colocalizes with myosin light chains in cardiac myocytes
brenda
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
LOCALIZATION
ORGANISM
UNIPROT
COMMENTARY
GeneOntology No.
LITERATURE
SOURCE
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
GENERAL INFORMATION
ORGANISM
UNIPROT
COMMENTARY
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
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
UNIPROT
ENTRY NAME
ORGANISM
NO. OF AA
NO. OF TRANSM. HELICES
MOLECULAR WEIGHT[Da]
SOURCE
Sequence
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
PDB
SCOP
CATH
UNIPROT
ORGANISM
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
MOLECULAR WEIGHT
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
55000
160000
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
SUBUNIT
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
homotrimer
trimer
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
pH STABILITY
ORGANISM
UNIPROT
COMMENTARY
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
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
TEMPERATURE STABILITY
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
4
-
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
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
STORAGE STABILITY
ORGANISM
UNIPROT
LITERATURE
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
PURIFICATION/commentary
ORGANISM
UNIPROT
LITERATURE
14.2fold to apparent homogeneity, by hydrophobic chromatography and affinity chromatography
-
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
-
using column chromatography on SP-Sepharose, Red Sepharose CL-6B and Hydroxylapatite
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
CLONED/commentary
ORGANISM
UNIPROT
LITERATURE
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
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
-
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
APPLICATION
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
diagnostics
-
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
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
REF.
AUTHORS
TITLE
JOURNAL
VOL.
PAGES
YEAR
ORGANISM (UNIPROT)
PUBMED ID
SOURCE
Hulse, J.D.; Henderson, L.M.
Carnitine biosynthesis. Purification of 4-N-trimethylaminobutyraldehyde dehydrogenase from beef liver
J. Biol. Chem.
255
1146-1151
1980
Bos taurus
brenda
Vaz, F.M.; Fouchier, S.W.; Ofman, R.; Sommer, M.; Wanders, R.J.A.
Molecular and biochemical characterization of rat gamma-trimethylaminobutyraldehyde dehydrogenase and evidence for the involvement of human aldehyde dehydrogenase 9 in carnitine biosynthesis
J. Biol. Chem.
275
7390-7394
2000
Rattus norvegicus (Q9JLJ3), Rattus norvegicus Wistar (Q9JLJ3)
brenda
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, Rattus norvegicus
brenda
Marchitti, S.A.; Deitrich, R.A.; Vasiliou, V.
Neurotoxicity and metabolism of the catecholamine-derived 3,4-dihydroxyphenylacetaldehyde and 3,4-dihydroxyphenylglycolaldehyde: the role of aldehyde dehydrogenase
Pharmacol. Rev.
59
125-150
2007
Homo sapiens (P49189)
brenda
Alnouti, Y.; Klaassen, C.D.
Tissue distribution, ontogeny, and regulation of aldehyde dehydrogenase (Aldh) enzymes mRNA by prototypical microsomal enzyme inducers in mice
Toxicol. Sci.
101
51-64
2008
Mus musculus (Q9JLJ2)
brenda
Hassan, M.; Okada, M.; Ichiyanagi, T.; Mori, N.
4-N-trimethylaminobutyraldehyde dehydrogenase: purification and characterization of an enzyme from Pseudomonas sp. 13CM
Biosci. Biotechnol. Biochem.
72
155-162
2008
Pseudomonas sp. 13CM, Pseudomonas sp.
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 (Q9JLJ3)
brenda
Wen, G.; Ringseis, R.; Rauer, C.; Eder, K.
The mouse gene encoding the carnitine biosynthetic enzyme 4-N-trimethylaminobutyraldehyde dehydrogenase is regulated by peroxisome proliferator-activated receptor alpha
Biochim. Biophys. Acta
1819
357-365
2012
Mus musculus, Mus musculus (Q9JLJ2)
brenda
Bari, M.R.; Hassan, M.; Akai, N.; Arima, J.; Mori, N.
Gene cloning and biochemical characterization of 4-N-trimethylaminobutyraldehyde dehydrogenase II from Pseudomonas sp. 13CM
World J. Microbiol. Biotechnol.
29
683-692
2013
Pseudomonas sp. 13CM (N0DT23), Pseudomonas sp. (N0DT23), Pseudomonas sp.
brenda
Matsunaga, A.; Harita, Y.; Shibagaki, Y.; Shimizu, N.; Shibuya, K.; Ono, H.; Kato, H.; Sekine, T.; Sakamoto, N.; Igarashi, T.; Hattori, S.
Identification of 4-trimethylaminobutyraldehyde dehydrogenase (TMABA-DH) as a candidate serum autoantibody target for Kawasaki disease
PLoS ONE
10
e0128189
2015
Homo sapiens, Homo sapiens (P49189), Rattus norvegicus (Q9JLJ3)
brenda
Select items on the left to see more content.
EXTERNAL LINKS (specific for EC-Number 1.2.1.47)
html completed
Use of this online version of BRENDA is free under the CC BY 4.0 license. See terms of use for full details.
Information
