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Information on EC 1.2.1.47 - 4-trimethylammoniobutyraldehyde dehydrogenase
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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
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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
-
-
-
-
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REACTION
REACTION DIAGRAM
COMMENTARY
ORGANISM
UNIPROT
LITERATURE
4-trimethylammoniobutanal + NAD+ + H2O = 4-trimethylammoniobutanoate + NADH + 2 H+
-
-
-
-
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REACTION TYPE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
oxidation
-
-
-
-
redox reaction
-
-
-
-
reduction
reduction
-
-
-
-
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PATHWAY SOURCE
PATHWAYS
BRENDA
KEGG
MetaCyc
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SYSTEMATIC NAME
IUBMB Comments
4-trimethylammoniobutanal:NAD+ 1-oxidoreductase
-
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CAS REGISTRY NUMBER
COMMENTARY
73361-01-0
-
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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
4-trimethylaminobutyraldehyde + NADP+ + H2O
4-trimethylaminobutyrate + NADPH + H+
-
-
-
r
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-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+
-
-
-
?
gamma-aminobutyraldehyde + NAD+
gamma-aminobutyrate + NADH
-
-
?
gamma-aminobutyraldehyde + NAD+
gamma-aminobutyrate + NADH
-
-
?
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
?
-
-
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
-
-
?
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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+
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+
-
-
-
?
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-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+
-
-
-
?
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COFACTOR
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
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METALS and IONS
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
additional information
additional information
Rb+, Na+, Mg2+, and Ca2+ have no effect on the enzyme activity
additional information
-
Rb+, Na+, Mg2+, and Ca2+ have no effect on the enzyme activity
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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
-
additional information
-
potassium fluoride, sodium azide, 2,2'-dipyridyl, and EDTA have no effect on the enzyme activity
-
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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.
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KM VALUE [mM]
SUBSTRATE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
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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
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
additional information
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
LITERATURE
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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
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TEMPERATURE OPTIMUM
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
40
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pI VALUE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
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ORGANISM
COMMENTARY
LITERATURE
UNIPROT
SEQUENCE DB
SOURCE
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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
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LOCALIZATION
ORGANISM
UNIPROT
COMMENTARY
GeneOntology No.
LITERATURE
SOURCE
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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
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UNIPROT
ENTRY NAME
ORGANISM
NO. OF AA
NO. OF TRANSM. HELICES
MOLECULAR WEIGHT[Da]
SOURCE
SEQUENCE
LOCALIZATION PREDICTION
The reliability class of the localization prediction ranges from 1 (very reliable) to 5 (not reliable).
The reliability class of the localization prediction ranges from 1 (very reliable) to 5 (not reliable). AL9A1_BOVIN
494
0
53977
Swiss-Prot
other Location (Reliability: 5)
AL9A1_GADMC
Gadus morhua subsp. callarias
503
0
54368
Swiss-Prot
other Location (Reliability: 1)
AL9A1_HUMAN
494
0
53802
Swiss-Prot
other Location (Reliability: 5)
AL9A1_MOUSE
494
0
53515
Swiss-Prot
other Location (Reliability: 5)
AL9A1_ORYLA
505
0
54482
Swiss-Prot
other Location (Reliability: 2)
AL9A1_PIG
494
0
53941
Swiss-Prot
other Location (Reliability: 4)
AL9A1_PONAB
494
0
53787
Swiss-Prot
other Location (Reliability: 5)
AL9A1_RAT
494
0
53653
Swiss-Prot
other Location (Reliability: 4)
A9A1A_DANRE
508
0
55262
Swiss-Prot
other Location (Reliability: 2)
A9A1B_DANRE
518
0
56438
Swiss-Prot
Mitochondrion (Reliability: 3)
B8CQM3_SHEPW
Shewanella piezotolerans (strain WP3 / JCM 13877)
487
0
52508
TrEMBL
-
A0A1Z5J825_FISSO
513
0
55708
TrEMBL
Mitochondrion (Reliability: 2)
A0A6J8D2F5_MYTCO
514
0
55733
TrEMBL
other Location (Reliability: 1)
A0A6J8D494_MYTCO
509
0
55247
TrEMBL
other Location (Reliability: 1)
A0A6J8CZP9_MYTCO
523
0
56627
TrEMBL
other Location (Reliability: 1)
A0A084FU31_PSEDA
496
0
54254
TrEMBL
other Location (Reliability: 2)
A0A084G3B9_PSEDA
497
0
53745
TrEMBL
other Location (Reliability: 2)
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PDB
SCOP
CATH
UNIPROT
ORGANISM
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MOLECULAR WEIGHT
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
160000
55000
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SUBUNIT
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
homotrimer
trimer
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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
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TEMPERATURE STABILITY
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
25 - 30
the enzyme is stable up to 25°C while incubation for 30 min at 30°C and above leads to rapid inactivation
4
-
0.1% 2-mercaptoethanol, 20% glycerol, fairly stable, but storage for several weeks results in loss of most of the activity
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STORAGE STABILITY
ORGANISM
UNIPROT
LITERATURE
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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
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CLONED (Commentary)
ORGANISM
UNIPROT
LITERATURE
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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
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
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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., Pseudomonas sp. 13CM
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 (Q9JLJ2), Mus musculus
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. (N0DT23), Pseudomonas sp., Pseudomonas sp. 13CM (N0DT23)
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 (P49189), Homo sapiens, Rattus norvegicus (Q9JLJ3)
brenda
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