Information on EC 1.5.5.1 - electron-transferring-flavoprotein dehydrogenase and Organism(s) Homo sapiens

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Homo sapiens


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


The taxonomic range for the selected organisms is: Homo sapiens

EC NUMBER
COMMENTARY hide
1.5.5.1
-
RECOMMENDED NAME
GeneOntology No.
electron-transferring-flavoprotein dehydrogenase
REACTION TYPE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
oxidation
-
-
-
-
redox reaction
-
-
-
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reduction
-
-
-
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PATHWAY
BRENDA Link
KEGG Link
MetaCyc Link
oxidative phosphorylation
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-
SYSTEMATIC NAME
IUBMB Comments
electron-transferring-flavoprotein:ubiquinone oxidoreductase
An iron-sulfur flavoprotein, forming part of the mitochondrial electron-transfer system.
CAS REGISTRY NUMBER
COMMENTARY hide
86551-03-3
-
ORGANISM
COMMENTARY hide
LITERATURE
UNIPROT
SEQUENCE DB
SOURCE
GENERAL INFORMATION
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
malfunction
physiological function
additional information
-
electron transfer flavoprotein structure analysis and FAD binding, overview
SUBSTRATE
PRODUCT                       
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate) hide
LITERATURE
(Substrate)
COMMENTARY
(Product) hide
LITERATURE
(Product)
Reversibility
r=reversible
ir=irreversible
?=not specified
reduced electron-transferring flavoprotein + 2,3-dimethoxy-5-methyl-6-(3-methylbut-2-en)-1,4-benzoquinone
electron-transferring flavoprotein + 2,3-dimethoxy-5-methyl-6-(3-methylbut-2-en)-1,4-benzoquinol
show the reaction diagram
-
-
-
?
reduced electron-transferring flavoprotein + 2,6-dichloroindophenol
electron-transferring flavoprotein + reduced 2,6-dichloroindophenol
show the reaction diagram
-
-
-
-
?
reduced electron-transferring flavoprotein + coenzyme Q1
electron-transferring flavoprotein + reduced coenzyme Q1
show the reaction diagram
-
-
-
-
?
reduced electron-transferring flavoprotein + decylubiquinone
electron-transferring flavoprotein + decylubiquinol
show the reaction diagram
-
-
-
-
?
reduced electron-transferring flavoprotein + ubiquinone
electron-transferring flavoprotein + ubiquinol
show the reaction diagram
reduced electron-transferring flavoprotein + ubiquinone-2
electron-transferring flavoprotein + ubiquinol-2
show the reaction diagram
reduced electron-transferring flavoprotein + ubiquinone-4
electron-transferring flavoprotein + ubiquinol-4
show the reaction diagram
-
-
-
-
?
reduced electron-transferring flavoprotein-4'-deoxy-FAD + ubiquinone-1
electron-transferring flavoprotein-4'-deoxy-FAD + ubiquinol-1
show the reaction diagram
-
0.07% of turnover with native electron-transferring flavoprotein
-
?
reduced electron-transferring-flavoprotein + 2,5-dibromo-3-methyl-6-isopropyl-4-benzoquinone
electron-transferring-flavoprotein + 2,5-dibromo-3-methyl-6-isopropyl-4-benzoquinol
show the reaction diagram
-
-
-
-
?
reduced electron-transferring-flavoprotein + 6-bis(isoprenyl)ubiquinone
electron-transferring-flavoprotein + 6-bis(isoprenyl)ubiquinol
show the reaction diagram
-
optimal ubiquinone derivative
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-
?
reduced electron-transferring-flavoprotein + 6-isoprenylubiquinone
electron-transferring-flavoprotein + 6-isoprenylubiquinol
show the reaction diagram
-
-
-
-
?
reduced electron-transferring-flavoprotein + bromodecylubiquinone
electron-transferring-flavoprotein + bromodecylubiquinol
show the reaction diagram
-
-
-
-
?
reduced electron-transferring-flavoprotein + decylubiquinone
electron-transferring-flavoprotein + decylubiquinol
show the reaction diagram
-
-
-
-
?
reduced electron-transferring-flavoprotein + duroquinone
electron-transferring-flavoprotein + duroquinol
show the reaction diagram
-
-
-
-
?
reduced electron-transferring-flavoprotein + heptylubiquinone
electron-transferring-flavoprotein + heptylubiquinol
show the reaction diagram
-
-
-
-
?
reduced electron-transferring-flavoprotein + hydroxydecylubiquinone
electron-transferring-flavoprotein + hydroxydecylubiquinol
show the reaction diagram
-
-
-
-
?
reduced electron-transferring-flavoprotein + menadione
electron-transferring-flavoprotein + menadiol
show the reaction diagram
-
-
-
-
?
reduced electron-transferring-flavoprotein + nonylubiquinone
electron-transferring-flavoprotein + nonylubiquinol
show the reaction diagram
-
-
-
-
?
reduced electron-transferring-flavoprotein + pentadecylubiquinone
electron-transferring-flavoprotein + pentadecylubiquinol
show the reaction diagram
-
-
-
-
?
reduced electron-transferring-flavoprotein + tridecylubiquinone
electron-transferring-flavoprotein + tridecylubiquinol
show the reaction diagram
-
-
-
-
?
reduced electron-transferring-flavoprotein + ubiquinone
electron-transferring-flavoprotein + ubiquinol
show the reaction diagram
additional information
?
-
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
reduced electron-transferring flavoprotein + ubiquinone
electron-transferring flavoprotein + ubiquinol
show the reaction diagram
reduced electron-transferring-flavoprotein + ubiquinone
electron-transferring-flavoprotein + ubiquinol
show the reaction diagram
-
ETF is the intermediate electron carrier between dehydrogenases and the enzyme, enzyme mediates between eleven mitochondrial proteins and the ubiquinone pool
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-
?
additional information
?
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Q16134
enzyme deficiency leads to glutaric acidemia type II
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COFACTOR
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
ubiquinone
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[4Fe-4S]-center
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-
METALS and IONS
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
4Fe-4S centre
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contains a single [4Fe-4S]2+,1+ cluster
Fe2+
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enzyme contains iron-sulfur clusters
INHIBITORS
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
2-(3-methylpentyl)-4,6-dinitrophenol
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; noncompetitive
2-n-heptyl-4-hydroxyquinoline N-oxide
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p-chloromercuribenzoate
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complete inhibition of ubiquinone reductase activity, 70% inhibition of disproportion activity
Pentachlorophenol
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; noncompetitive
additional information
-
inhibition mechanism
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KM VALUE [mM]
SUBSTRATE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
0.0069
6-bis(isoprenyl)ubiquinone
-
recombinant enzyme, pH 7.4, 25C
0.0095
6-isoprenylubiquinone
-
recombinant enzyme, pH 7.4, 25C
0.0089
bromodecylubiquinone
-
recombinant enzyme, pH 7.4, 25C
0.0079 - 0.0084
decylubiquinone
0.0195
duroquinone
-
recombinant enzyme, pH 7.4, 25C
0.00013 - 0.0113
electron-transferring flavoprotein
0.0082
heptylubiquinone
-
recombinant enzyme, pH 7.4, 25C
0.0099
hydroxydecylubiquinone
-
recombinant enzyme, pH 7.4, 25C
0.0163
menadione
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recombinant enzyme, pH 7.4, 25C
0.0073
nonylubiquinone
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recombinant enzyme, pH 7.4, 25C
0.0023
pentadecylubiquinone
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recombinant enzyme, pH 7.4, 25C
0.0154
semiquinone electron-transferring flavoprotein
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0.0265
tridecylubiquinone
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recombinant enzyme, pH 7.4, 25C
0.0081
ubiquinone-1
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0.0049
ubiquinone-2
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0.0148
ubiquinone-4
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additional information
additional information
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substrate binding and steady-state reaction kinetics
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TURNOVER NUMBER [1/s]
SUBSTRATE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
68.7
6-bis(isoprenyl)ubiquinone
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recombinant enzyme, pH 7.4, 25C
34.5
6-isoprenylubiquinone
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recombinant enzyme, pH 7.4, 25C
60.8
bromodecylubiquinone
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recombinant enzyme, pH 7.4, 25C
59.9 - 74.3
decylubiquinone
18.1
duroquinone
-
recombinant enzyme, pH 7.4, 25C
21.4
electron-transferring flavoprotein
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54
heptylubiquinone
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recombinant enzyme, pH 7.4, 25C
67
hydroxydecylubiquinone
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recombinant enzyme, pH 7.4, 25C
6.6
menadione
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recombinant enzyme, pH 7.4, 25C
61.3
nonylubiquinone
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recombinant enzyme, pH 7.4, 25C
7.4
pentadecylubiquinone
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recombinant enzyme, pH 7.4, 25C
81.4
semiquinone electron-transferring flavoprotein
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31.5
tridecylubiquinone
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recombinant enzyme, pH 7.4, 25C
78.8
ubiqinone-2
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33.5
ubiquinone-1
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35.5
ubiquinone-4
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Ki VALUE [mM]
INHIBITOR
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
0.0046
2-(3-methylpentyl)-4,6-dinitrophenol
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recombinant enzyme, pH 7.4, 25C
0.0077
Pentachlorophenol
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recombinant enzyme, pH 7.4, 25C
SPECIFIC ACTIVITY [µmol/min/mg]
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
0.139
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ubiquinone reduction
0.21
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deletion mutant enzyme lacking 74 amino acids, disproportionation of semiquinone electron-transferring flavoprotein
0.33
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C561A mutant enzyme, disproportionation of semiquinone electron-transferring flavoprotein
0.68
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disproportionation of semiquinone electron-transferring flavoprotein
1.3
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disproportionation of semiquinone electron-transferring flavoprotein
17.6
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recombinant enzyme, ubiquinone-1 reduction
additional information
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-
pH OPTIMUM
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
7.2
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assay at
7.4
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assay at
TEMPERATURE OPTIMUM
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
22
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assay at room temperature
25
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assay at
SOURCE TISSUE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
SOURCE
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from thigh and limb
Manually annotated by BRENDA team
LOCALIZATION
ORGANISM
UNIPROT
COMMENTARY hide
GeneOntology No.
LITERATURE
SOURCE
MOLECULAR WEIGHT
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
67000
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x * 67000, immunoprecipitation after in vitro translation, deduced from nucletide sequence
SUBUNITS
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
Purification/COMMENTARY
ORGANISM
UNIPROT
LITERATURE
recombinant enzyme from Sf9 insect cells
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recombinant enzyme, DEAE-Sepharose, HA-Ultrogel, Mono Q
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recombinant N-terminally His6-tagged enzyme from Escherichia coli strain BL21 CodonPlus(DE3)-RIPL by nickel affinity chromatography in presence of FAD
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Cloned/COMMENTARY
ORGANISM
UNIPROT
LITERATURE
deletion mutant enzyme lacking 74 amino acids among them C561, and a C561A mutant
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expressed from a baculovirus vector, expressed in Sf9 cells
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expression in insect Sf9 cells via transfection using the baculovirus system
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expression in Saccharomyces cerevisiae, the enzyme is synthesized as a 67000 Da precursor which is targeted to mitochondria and processed in a single step to a 64000 Da mature form
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expression in Sf9 insect cells
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gene ETF:QO, DNA and amino acid sequence determination and analysis, localization on chromosome 4q33, 13 exons
recombinant expression of N-terminally His6-tagged enzyme in Escherichia coli strain BL21 CodonPlus(DE3)-RIPL
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ENGINEERING
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
C561A
-
mutant enzyme has no ubiquinone reductase activity
D218N
heterozygous, with a deletion on the other allele, naturally occurring mutation of gene ETF:QO in patients with glutaric acidemia type II, no antigen detected in fibroblasts
G611E
homozygous, naturally occurring mutation of gene ETF:QO in patients with glutaric acidemia type II, no antigen detected in fibroblasts
I31T
neutral naturally occurring mutation of gene ETF:QO in patients with glutaric acidemia type II, no effect on enzyme activity or expression, occurs together with other mutantions, overview
L262F
homozygous, naturally occurring mutation of gene ETF:QO in patients with glutaric acidemia type II, no antigen detected in fibroblasts
L334P
homo- or heterozygous, the latter with a deletion on the other allele, naturally occurring mutation of gene ETF:QO in patients with glutaric acidemia type II, reduced antigen detected in fibroblasts
L334P/Q222P
mutations on different alleles, naturally occurring mutations of gene ETF:QO in patients with glutaric acidemia type II
L377P
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the mutation is involved in the myopathic form of CoQ10 deficiency
M1T
homo- and heterozygous, the latter with a deletion on the other allele, naturally occurring mutation of gene ETF:QO in patients with glutaric acidemia type II, no antigen detected in fibroblasts
P456L
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the mutation affects most likely the catalytic activity and the stability of the tetramer
P483L
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the mutation affects most likely the catalytic activity and the stability of the tetramer
P562L
heterozygous, with a deletion on the other allele, naturally occurring mutation of gene ETF:QO in patients with glutaric acidemia type II, no antigen detected in fibroblasts
R41X/L138R
mutations on different alleles, naturally occurring mutations of gene ETF:QO in patients with glutaric acidemia type II
R452K
homozygous, naturally occurring mutation of gene ETF:QO in patients with glutaric acidemia type II
S82F/D218N
mutations on different alleles, naturally occurring mutations of gene ETF:QO in patients with glutaric acidemia type II, reduced antigen detected in fibroblasts
S82P/H346R
mutations on different alleles, naturally occurring mutations of gene ETF:QO in patients with glutaric acidemia type II, no antigen detected in fibroblasts
W182X/P456L
mutations on different alleles, naturally occurring mutations of gene ETF:QO in patients with glutaric acidemia type II, reduced antigen detected in fibroblasts
Y49C
heterozygous, naturally occurring mutation of gene ETF:QO in patients with glutaric acidemia type II, no antigen detected in fibroblasts
additional information
determination and analysis of diverse naturally occurring mutations of gene ETF:QO in patients with glutaric acidemia type II, phenotypic effects, overview
APPLICATION
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
medicine
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mutations in the ETFDH gene lead to a secondary myopathic form of CoQ10 deficiency and to the late-onset form of glutaric aciduria type II