BRENDA - Enzyme Database show
show all sequences of 1.14.11.8

Functional analysis of TMLH variants and definition of domains required for catalytic activity and mitochondrial targeting

Monfregola, J.; Cevenini, A.; Terracciano, A.; van Vlies, N.; Arbucci, S.; Wanders, R.J.; DUrso, M.; Vaz, F.M.; Ursini, M.V.; J. Cell. Physiol. 204, 839-847 (2005)

Data extracted from this reference:

Activating Compound
Activating Compound
Commentary
Organism
Structure
2-oxoglutarate
dependent
Homo sapiens
2-oxoglutarate
dependent
Mus musculus
Fe2+
dependent
Homo sapiens
Fe2+
dependent
Mus musculus
Cloned(Commentary)
Commentary
Organism
expressed in COS-1 cells
Homo sapiens
expressed in COS-1 cells
Mus musculus
Engineering
Amino acid exchange
Commentary
Organism
H389L
enzyme not functional
Homo sapiens
H389L
enzyme not functional
Mus musculus
Localization
Localization
Commentary
Organism
GeneOntology No.
Textmining
mitochondrion
-
Mus musculus
5739
-
mitochondrion
-
Homo sapiens
5739
-
Organism
Organism
Primary Accession No. (UniProt)
Commentary
Textmining
Homo sapiens
-
-
-
Mus musculus
-
-
-
Source Tissue
Source Tissue
Commentary
Organism
Textmining
brain
-
Homo sapiens
-
brain
-
Mus musculus
-
heart
-
Homo sapiens
-
heart
-
Mus musculus
-
intestine
-
Homo sapiens
-
kidney
-
Homo sapiens
-
kidney
-
Mus musculus
-
liver
-
Homo sapiens
-
liver
-
Mus musculus
-
muscle
-
Homo sapiens
-
muscle
-
Mus musculus
-
placenta
-
Homo sapiens
-
testis
-
Mus musculus
-
Substrates and Products (Substrate)
Substrates
Commentary Substrates
Literature (Substrates)
Organism
Products
Commentary (Products)
Literature (Products)
Organism (Products)
Reversibility
N6,N6,N6-trimethyl-L-lysine + 2-oxoglutarate + O2
-
675029
Mus musculus
3-hydroxy-N6,N6,N6-trimethyl-L-lysine + succinate + CO2
-
-
-
?
N6,N6,N6-trimethyl-L-lysine + 2-oxoglutarate + O2
-
675029
Homo sapiens
3-hydroxy-N6,N6,N6-trimethyl-L-lysine + succinate + CO2
-
-
-
?
Activating Compound (protein specific)
Activating Compound
Commentary
Organism
Structure
2-oxoglutarate
dependent
Homo sapiens
2-oxoglutarate
dependent
Mus musculus
Fe2+
dependent
Homo sapiens
Fe2+
dependent
Mus musculus
Cloned(Commentary) (protein specific)
Commentary
Organism
expressed in COS-1 cells
Homo sapiens
expressed in COS-1 cells
Mus musculus
Engineering (protein specific)
Amino acid exchange
Commentary
Organism
H389L
enzyme not functional
Homo sapiens
H389L
enzyme not functional
Mus musculus
Localization (protein specific)
Localization
Commentary
Organism
GeneOntology No.
Textmining
mitochondrion
-
Homo sapiens
5739
-
mitochondrion
-
Mus musculus
5739
-
Source Tissue (protein specific)
Source Tissue
Commentary
Organism
Textmining
brain
-
Homo sapiens
-
brain
-
Mus musculus
-
heart
-
Homo sapiens
-
heart
-
Mus musculus
-
intestine
-
Homo sapiens
-
kidney
-
Homo sapiens
-
kidney
-
Mus musculus
-
liver
-
Homo sapiens
-
liver
-
Mus musculus
-
muscle
-
Homo sapiens
-
muscle
-
Mus musculus
-
placenta
-
Homo sapiens
-
testis
-
Mus musculus
-
Substrates and Products (Substrate) (protein specific)
Substrates
Commentary Substrates
Literature (Substrates)
Organism
Products
Commentary (Products)
Literature (Products)
Organism (Products)
Reversibility
N6,N6,N6-trimethyl-L-lysine + 2-oxoglutarate + O2
-
675029
Mus musculus
3-hydroxy-N6,N6,N6-trimethyl-L-lysine + succinate + CO2
-
-
-
?
N6,N6,N6-trimethyl-L-lysine + 2-oxoglutarate + O2
-
675029
Homo sapiens
3-hydroxy-N6,N6,N6-trimethyl-L-lysine + succinate + CO2
-
-
-
?
Other publictions for EC 1.14.11.8
No.
1st author
Pub Med
title
organims
journal
volume
pages
year
Activating Compound
Application
Cloned(Commentary)
Crystallization (Commentary)
Engineering
General Stability
Inhibitors
KM Value [mM]
Localization
Metals/Ions
Molecular Weight [Da]
Natural Substrates/ Products (Substrates)
Organic Solvent Stability
Organism
Oxidation Stability
Posttranslational Modification
Purification (Commentary)
Reaction
Renatured (Commentary)
Source Tissue
Specific Activity [micromol/min/mg]
Storage Stability
Substrates and Products (Substrate)
Subunits
Temperature Optimum [°C]
Temperature Range [°C]
Temperature Stability [°C]
Turnover Number [1/s]
pH Optimum
pH Range
pH Stability
Cofactor
Ki Value [mM]
pI Value
IC50 Value
Activating Compound (protein specific)
Application (protein specific)
Cloned(Commentary) (protein specific)
Cofactor (protein specific)
Crystallization (Commentary) (protein specific)
Engineering (protein specific)
General Stability (protein specific)
IC50 Value (protein specific)
Inhibitors (protein specific)
Ki Value [mM] (protein specific)
KM Value [mM] (protein specific)
Localization (protein specific)
Metals/Ions (protein specific)
Molecular Weight [Da] (protein specific)
Natural Substrates/ Products (Substrates) (protein specific)
Organic Solvent Stability (protein specific)
Oxidation Stability (protein specific)
Posttranslational Modification (protein specific)
Purification (Commentary) (protein specific)
Renatured (Commentary) (protein specific)
Source Tissue (protein specific)
Specific Activity [micromol/min/mg] (protein specific)
Storage Stability (protein specific)
Substrates and Products (Substrate) (protein specific)
Subunits (protein specific)
Temperature Optimum [°C] (protein specific)
Temperature Range [°C] (protein specific)
Temperature Stability [°C] (protein specific)
Turnover Number [1/s] (protein specific)
pH Optimum (protein specific)
pH Range (protein specific)
pH Stability (protein specific)
pI Value (protein specific)
Expression
General Information
General Information (protein specific)
Expression (protein specific)
KCat/KM [mM/s]
KCat/KM [mM/s] (protein specific)
744681
Lesniak
Human carnitine biosynthesis ...
Homo sapiens
Chem. Commun. (Camb.)
53
440-442
2017
-
-
1
-
-
-
-
-
1
1
-
1
-
1
-
-
1
-
-
-
-
-
1
-
-
-
-
-
1
-
-
-
-
-
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-
-
1
-
-
-
-
-
-
-
-
1
1
-
1
-
-
-
1
-
-
-
-
1
-
-
-
-
-
1
-
-
-
-
2
2
-
-
-
745958
Vijayendar Reddy
Fluorinated trimethyllysine a ...
Homo sapiens
Org. Biomol. Chem.
15
1350-1354
2017
1
-
-
-
-
-
-
-
-
1
-
1
-
1
-
-
-
-
-
-
-
-
3
-
1
-
-
-
1
-
-
-
-
-
-
1
-
-
-
-
-
-
-
-
-
-
-
1
-
1
-
-
-
-
-
-
-
-
3
-
1
-
-
-
1
-
-
-
-
2
2
-
-
-
745961
Reddy
Evidence that trimethyllysine ...
Homo sapiens
Org. Lett.
19
400-403
2017
-
-
-
-
-
-
-
-
1
1
-
1
-
2
-
-
-
-
-
-
-
-
2
-
1
-
-
-
1
-
-
-
-
-
-
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-
-
-
-
-
-
-
-
-
-
1
1
-
1
-
-
-
-
-
-
-
-
2
-
1
-
-
-
1
-
-
-
-
2
2
-
-
-
744678
Al Temimi
Substrate scope for trimethyl ...
Homo sapiens
Chem. Commun. (Camb.)
52
12849-12852
2016
1
-
-
-
-
-
-
-
-
1
-
1
-
2
-
-
-
-
-
-
-
-
7
-
1
-
-
-
1
-
-
-
-
-
-
1
-
-
-
-
-
-
-
-
-
-
-
1
-
1
-
-
-
-
-
-
-
-
7
-
1
-
-
-
1
-
-
-
-
4
4
-
-
-
746366
Kazaks
Expression and purification o ...
Homo sapiens
Protein Expr. Purif.
104
1-6
2014
1
-
1
-
-
-
-
3
-
1
-
1
-
2
-
-
1
-
-
-
-
1
2
1
1
-
-
-
1
-
-
-
-
-
-
1
-
1
-
-
-
-
-
-
-
3
-
1
-
1
-
-
-
1
-
-
-
1
2
1
1
-
-
-
1
-
-
-
-
1
1
-
-
-
697027
Fischer
A moderate excess of dietary l ...
Sus scrofa
Br. J. Nutr.
101
190-196
2009
-
-
-
-
-
-
-
-
1
-
-
1
-
2
-
-
-
-
-
4
-
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1
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1
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1
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4
-
-
1
-
-
-
-
-
-
-
-
-
1
1
1
1
-
-
691166
Furusawa
Vitamin C is not essential for ...
Mus musculus
Biol. Pharm. Bull.
31
1673-1679
2008
1
-
-
-
1
-
-
-
1
-
-
1
-
2
-
-
-
-
-
-
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-
1
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-
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-
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1
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1
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1
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1
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1
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-
-
-
-
-
-
-
-
1
1
-
-
-
686678
van Vlies
Submitochondrial localization ...
Rattus norvegicus
FEBS J.
274
5845-5851
2007
-
-
-
-
-
-
-
-
1
-
-
1
-
1
-
-
-
-
-
1
-
-
1
-
-
-
-
-
-
-
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1
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1
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1
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1
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
671302
van Vlies
Measurement of carnitine biosy ...
Mus musculus, Rattus norvegicus
Anal. Biochem.
354
132-139
2006
-
-
-
-
-
-
2
-
-
2
-
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2
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8
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2
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2
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2
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2
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2
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8
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2
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-
-
-
-
675029
Monfregola
Functional analysis of TMLH va ...
Homo sapiens, Mus musculus
J. Cell. Physiol.
204
839-847
2005
4
-
2
-
2
-
-
-
2
-
-
-
-
3
-
-
-
-
-
13
-
-
2
-
-
-
-
-
-
-
-
-
-
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-
4
-
2
-
-
2
-
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2
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-
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-
-
13
-
-
2
-
-
-
-
-
-
-
-
-
-
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-
-
-
-
675561
Davis
Carnitine deficiency and suppl ...
Rattus norvegicus
J. Nutr.
135
761-764
2005
-
-
-
-
-
-
-
-
-
-
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2
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2
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1
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2
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1
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651370
Swiegers
Carnitine biosynthesis in neur ...
Neurospora crassa
FEMS Microbiol. Lett.
210
19-23
2002
-
-
-
-
-
-
-
2
-
1
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3
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1
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1
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652164
Vaz
Molecular and biochemical char ...
Homo sapiens, Mus musculus, Rattus norvegicus
J. Biol. Chem.
276
33512-33517
2001
-
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2
-
-
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2
1
1
2
1
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7
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1
1
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1
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3
1
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1
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2
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2
1
1
2
1
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1
1
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1
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3
1
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1
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7020
Henderson
Mammalian enzymes of trimethyl ...
Bos taurus
Fed. Proc.
41
2843-2847
1982
1
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-
-
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9
2
1
2
3
-
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1
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1
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2
1
1
1
1
1
-
1
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1
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1
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1
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1
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9
-
2
1
2
3
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1
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2
1
1
1
1
1
-
1
-
1
-
-
-
-
-
-
-
-
-
7021
Sachan
Carnitine biosynthesis. Hydrox ...
Rattus norvegicus
Biochem. J.
188
529-534
1980
-
-
-
-
-
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3
1
1
2
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1
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1
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1
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1
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1
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3
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1
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2
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1
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1
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7022
Sachan
Synthesis of carnitine from ep ...
Neurospora crassa
Biochem. Biophys. Res. Commun.
96
870-875
1980
-
-
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1
1
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1
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1
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7019
Hulse
Carnitine biosynthesis. beta-H ...
Rattus norvegicus
J. Biol. Chem.
253
1654-1659
1978
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2
1
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1
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