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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
aldh9a1, tmaba-dh, 4-n-trimethylaminobutyraldehyde dehydrogenase, 4-trimethylaminobutyraldehyde dehydrogenase, gamma-trimethylaminobutyraldehyde dehydrogenase, tmabaldehyde-dh ii, carnitine biosynthesis enzyme, tmabadh, aldehyde dehydrogenase 9a1, 
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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
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-
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gamma-trimethylaminobutyraldehyde dehydrogenase
TMABA dehydrogenase
TMABA-DH
TMABaldehyde-DH II
4-N-trimethylaminobutyraldehyde dehydrogenase
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-
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4-trimethylaminobutyraldehyde dehydrogenase
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-
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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
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redox reaction
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reduction
reduction
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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
3-aminopropionaldehyde + NAD+ + H2O
3-aminopropionate + NADH + H+
-
-
-
?
3-guanidinopropionaldehyde + NAD+ + H2O
3-guanidinopropionate + NADH + H+
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-
-
?
4-(dimethylamino)butyraldehyde + NAD+ + H2O
4-(dimethylamino)butyrate + NADH + 2 H+
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-
-
?
4-(trimethylamino)butyraldehyde + NAD+ + H2O
4-(trimethylamino)butyrate + NADH + 2 H+
preferred substrate
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-
?
4-amino-2-hydroxybutyraldehyde + NAD+ + H2O
4-amino-2-hydroxybutyrate + NADH + H+
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-
-
?
4-guanidinobutyraldehyde + NAD+ + H2O
4-guanidinobutyrate + NADH + H+
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-
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?
4-trimethylaminobutyraldehyde + NADP+ + H2O
4-trimethylaminobutyrate + NADPH + H+
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-
-
r
gamma-aminobutyraldehyde + NAD+ + H2O
4-aminobutanoate + NADH + H+
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-
-
?
N,N,N-trimethyl-3-aminopropionaldehyde + NAD+ + H2O
N,N,N-trimethyl-3-aminopropionate + NADH + H+
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-
-
?
N,N-dimethyl-3-aminopropionaldehyde + NAD+ + H2O
N,N-dimethyl-3-aminopropionate + NADH + H+
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?
trimethylaminobutyraldehyde + NAD+ + H2O
gamma-butyrobetaine + NADH + 2 H+
enzyme is involved in L-carnitine (3-hydroxy-4-N-trimethylaminobutyrate) biosynthesis
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-
?
3,4-dihydroxyphenylacetaldehyde + NAD+ + H2O
3,4-dihydroxyphenylacetate + NADH + H+
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?
3,4-dihydroxyphenylacetaldehyde + NAD+ + H2O
3,4-dihydroxyphenylacetate + NADH + H+
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?
3,4-dihydroxyphenylacetaldehyde + NAD+ + H2O
3,4-dihydroxyphenylacetate + NADH + H+
a dopamine metabolite
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?
4-dimethylaminobutyraldehyde + NAD+
4-dimethylaminobutanoate + NADH
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?
4-dimethylaminobutyraldehyde + NAD+
4-dimethylaminobutanoate + NADH
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?
4-N-trimethylaminobutyraldehyde + NAD+
4-N-trimethylaminobutanoate + NADH
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?
4-N-trimethylaminobutyraldehyde + NAD+
4-N-trimethylaminobutanoate + NADH
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?
4-N-trimethylaminobutyraldehyde + NAD+ + H2O
gamma-butyrobetaine + NADH + H+
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?
4-N-trimethylaminobutyraldehyde + NAD+ + H2O
gamma-butyrobetaine + NADH + H+
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?
4-N-trimethylaminobutyraldehyde + NAD+ + H2O
gamma-butyrobetaine + NADH + H+
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?
4-trimethylaminobutyraldehyde + NAD+
4-trimethylaminobutanoate + NADH + H+
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?
4-trimethylaminobutyraldehyde + NAD+
4-trimethylaminobutanoate + NADH + H+
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?
4-trimethylaminobutyraldehyde + NAD+
4-trimethylaminobutyrate + NADH + H+
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?
4-trimethylaminobutyraldehyde + NAD+
4-trimethylaminobutyrate + NADH + H+
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function in carnitine biosynthesis
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?
4-trimethylaminobutyraldehyde + NAD+
4-trimethylaminobutyrate + NADH + H+
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?
4-trimethylaminobutyraldehyde + NAD+
4-trimethylaminobutyrate + NADH + H+
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?
4-trimethylaminobutyraldehyde + NAD+ + H2O
4-N-trimethylaminobutyrate + NADH + H+
strongly preferred substrate
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?
4-trimethylaminobutyraldehyde + NAD+ + H2O
4-N-trimethylaminobutyrate + NADH + H+
a carnitine precursor
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?
4-trimethylammoniobutanal + NAD+ + H2O
4-trimethylammoniobutanoate + NADH + 2 H+
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?
4-trimethylammoniobutanal + NAD+ + H2O
4-trimethylammoniobutanoate + NADH + 2 H+
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?
4-trimethylammoniobutanal + NAD+ + H2O
4-trimethylammoniobutanoate + NADH + 2 H+
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?
4-trimethylammoniobutanal + NAD+ + H2O
4-trimethylammoniobutanoate + NADH + 2 H+
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?
aminobutyraldehyde + NAD+ + H2O
aminobutyrate + NADH + H+
a GABA precursor
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?
gamma-aminobutyraldehyde + NAD+
gamma-aminobutyrate + NADH
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?
gamma-aminobutyraldehyde + NAD+
gamma-aminobutyrate + NADH
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?
additional information
?
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ALDH9A1 exhibits wide substrate specificity to aminoaldehydes, aliphatic and aromatic aldehydes with a clear preference for gamma-trimethylaminobutyraldehyde (TMABAL). The enzyme is multifunctional also catalyzing the reactions of EC 1.2.1.3 and EC 1.2.1.19. Substrate specificity, overview
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additional information
?
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ALDH9A1 exhibits wide substrate specificity to aminoaldehydes, aliphatic and aromatic aldehydes with a clear preference for gamma-trimethylaminobutyraldehyde (TMABAL). The enzyme is multifunctional also catalyzing the reactions of EC 1.2.1.3 and EC 1.2.1.19. Substrate specificity, overview
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additional information
?
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diethylaminobenzaldehyde (DEAB) is not a substrate for ALDH9A1
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additional information
?
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diethylaminobenzaldehyde (DEAB) is not a substrate for ALDH9A1
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additional information
?
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does not degradate 4-trimethylaminobutanol, betaine aldehyde, 4-aminobutyraldehyde, acetaldehyde, propionaldehyde, butyraldehyde, trimethylacetaldehyde, and 3-methylbutyraldehyde
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?
additional information
?
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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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?
additional information
?
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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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?
additional information
?
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does not degradate 4-trimethylaminobutanol, betaine aldehyde, 4-aminobutyraldehyde, acetaldehyde, propionaldehyde, butyraldehyde, trimethylacetaldehyde, and 3-methylbutyraldehyde
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?
additional information
?
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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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?
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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
3,4-dihydroxyphenylacetaldehyde + NAD+ + H2O
3,4-dihydroxyphenylacetate + NADH + H+
a dopamine metabolite
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?
4-(trimethylamino)butyraldehyde + NAD+ + H2O
4-(trimethylamino)butyrate + NADH + 2 H+
preferred substrate
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?
4-trimethylaminobutyraldehyde + NAD+ + H2O
4-N-trimethylaminobutyrate + NADH + H+
a carnitine precursor
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?
aminobutyraldehyde + NAD+ + H2O
aminobutyrate + NADH + H+
a GABA precursor
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-
?
trimethylaminobutyraldehyde + NAD+ + H2O
gamma-butyrobetaine + NADH + 2 H+
enzyme is involved in L-carnitine (3-hydroxy-4-N-trimethylaminobutyrate) biosynthesis
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-
?
4-N-trimethylaminobutyraldehyde + NAD+ + H2O
gamma-butyrobetaine + NADH + H+
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?
4-N-trimethylaminobutyraldehyde + NAD+ + H2O
gamma-butyrobetaine + NADH + H+
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-
-
?
4-N-trimethylaminobutyraldehyde + NAD+ + H2O
gamma-butyrobetaine + NADH + H+
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?
4-trimethylaminobutyraldehyde + NAD+
4-trimethylaminobutanoate + NADH + H+
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-
-
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?
4-trimethylaminobutyraldehyde + NAD+
4-trimethylaminobutanoate + NADH + H+
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-
-
-
?
4-trimethylaminobutyraldehyde + NAD+
4-trimethylaminobutyrate + NADH + H+
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function in carnitine biosynthesis
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?
4-trimethylaminobutyraldehyde + NAD+
4-trimethylaminobutyrate + NADH + H+
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?
4-trimethylaminobutyraldehyde + NAD+
4-trimethylaminobutyrate + NADH + H+
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-
?
4-trimethylammoniobutanal + NAD+ + H2O
4-trimethylammoniobutanoate + NADH + 2 H+
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?
4-trimethylammoniobutanal + NAD+ + H2O
4-trimethylammoniobutanoate + NADH + 2 H+
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?
4-trimethylammoniobutanal + NAD+ + H2O
4-trimethylammoniobutanoate + NADH + 2 H+
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?
4-trimethylammoniobutanal + NAD+ + H2O
4-trimethylammoniobutanoate + NADH + 2 H+
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-
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?
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COFACTOR
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
NAD+
enzyme binding structure, enzyme-NAD+ crystal structure analysis, overview. NAD+ binds in the expected site at the C-termini of the beta-strands of the Rossmann fold. NAD+ forms several electrostatic interactions with the protein. The adenine ribose hydrogen bonds with Lys180. The diphosphate interacts with Trp156, Ser233, and Thr236. The nicotinamide ribose of the one complete NAD+ forms a hydrogen bond with Glu391, a residue identically conserved in the ALDH superfamily
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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
diethylaminobenzaldehyde
DEAB, the broad-spectrum ALDH inhibitor reversibly inhibits ALDH9A1 in a time-dependent manner by a covalent reversible mechanism of inhibition, mechanism analysis, overview
diethylmethylamine
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at 30°C, 5 mM causes 53% inhibition, does not inhibit at 1 mM
additional information
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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
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additional information
potassium fluoride, sodium azide, 2,2'-dipyridyl, and EDTA have no effect on the enzyme activity
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additional information
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potassium fluoride, sodium azide, 2,2'-dipyridyl, and EDTA have no effect on the enzyme activity
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ACTIVATING COMPOUND
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
additional information
the presence of an aldehyde substrate and NAD+ promotes isomerization of the enzyme into the active conformation
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additional information
-
the presence of an aldehyde substrate and NAD+ promotes isomerization of the enzyme into the active conformation
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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
0.011
3,4-dihydroxyphenylacetaldehyde
pH 7.5, temperature not specified in the publication
0.056
3-aminopropionaldehyde
pH 7.5, temperature not specified in the publication
0.021
4-(dimethylamino)butyraldehyde
pH 7.5, temperature not specified in the publication
-
0.006
4-(trimethylamino)butyraldehyde
pH 7.5, temperature not specified in the publication
-
0.021
4-guanidinobutyraldehyde
pH 7.5, temperature not specified in the publication
0.053
N,N,N-trimethyl-3-aminopropionaldehyde
pH 7.5, temperature not specified in the publication
0.021
N,N-dimethyl-3-aminopropionaldehyde
pH 7.5, temperature not specified in the publication
-
0.067
4-Aminobutyraldehyde
pH 7.5, temperature not specified in the publication