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Information on EC 1.13.11.18 - persulfide dioxygenase and Organism(s) Homo sapiens

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EC Tree
IUBMB Comments
An iron protein. Perthiols, formed spontaneously by interactions between thiols and elemental sulfur or sulfide, are the only acceptable substrate to the enzyme. The sulfite that is formed by the enzyme can be further converted into sulfate, thiosulfate or S-sulfoglutathione (GSSO3-) non-enzymically .
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Homo sapiens
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Word Map
The taxonomic range for the selected organisms is: Homo sapiens
The expected taxonomic range for this enzyme is: Archaea, Bacteria, Eukaryota
Reaction Schemes
hide(Overall reactions are displayed. Show all >>)
Synonyms
sdo, ethe1, sulfur dioxygenase, persulfide dioxygenase, sulfur oxygenase, afe_0269, fe(ii)-containing persulfide dioxygenase, more
SYNONYM
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
ETHE1
sulfur oxygenase
-
-
-
-
REACTION TYPE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
oxidation
-
-
-
-
redox reaction
-
-
-
-
reduction
-
-
-
-
SYSTEMATIC NAME
IUBMB Comments
S-sulfanylglutathione:oxygen oxidoreductase
An iron protein. Perthiols, formed spontaneously by interactions between thiols and elemental sulfur or sulfide, are the only acceptable substrate to the enzyme. The sulfite that is formed by the enzyme can be further converted into sulfate, thiosulfate or S-sulfoglutathione (GSSO3-) non-enzymically [2].
CAS REGISTRY NUMBER
COMMENTARY hide
37256-58-9
-
SUBSTRATE
PRODUCT                       
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate) hide
LITERATURE
(Substrate)
COMMENTARY
(Product) hide
LITERATURE
(Product)
Reversibility
r=reversible
ir=irreversible
?=not specified
gamma-glutamyl-homocysteinyl-glycine + O2
gamma-glutamyl-homocysteinyl-SO2H + ?
show the reaction diagram
mechanism-based inhibitor, accepted as an alternative substrate and converted to glutamyl-homocysteinyl-SO2H
-
-
?
S-sulfanylglutathione + O2 + H2O
glutathione + sulfite + 2 H+
show the reaction diagram
-
-
-
?
METALS and IONS
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
Iron
iron content of wild-type is 0.8 mol/mol of enzyme
INHIBITOR
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
gamma-glutamyl-homocysteinyl-glycine
mechanism-based inhibitor, accepted as an alternative substrate and converted to glutamyl-homocysteinyl-SO2H, which fails to undergo the final hydrolysis step in the catalytic cycle, leading to inhibition. Maximum inhibition to 30% of initial activity
-
KM VALUE [mM]
SUBSTRATE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
0.34 - 1.2
S-sulfanylglutathione
TURNOVER NUMBER [1/s]
SUBSTRATE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
0.8 - 47
S-sulfanylglutathione
kcat/KM VALUE [1/mMs-1]
SUBSTRATE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
0.7 - 140
S-sulfanylglutathione
SPECIFIC ACTIVITY [µmol/min/mg]
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
113
wild-type, pH 7.4, 22°C
ORGANISM
COMMENTARY hide
LITERATURE
UNIPROT
SEQUENCE DB
SOURCE
LOCALIZATION
ORGANISM
UNIPROT
COMMENTARY hide
GeneOntology No.
LITERATURE
SOURCE
GENERAL INFORMATION
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
malfunction
-
ethylmalonic encephalopathy is caused by mutations in the mitochondrial matrix sulfur dioxygenase ETHE1 leading to failure to detoxify sulfide, a product of intestinal anaerobes and, in trace amounts, tissues
UNIPROT
ENTRY NAME
ORGANISM
NO. OF AA
NO. OF TRANSM. HELICES
MOLECULAR WEIGHT[Da]
SOURCE
SEQUENCE
LOCALIZATION PREDICTION?
ETHE1_HUMAN
254
0
27873
Swiss-Prot
Mitochondrion (Reliability: 2)
PDB
SCOP
CATH
UNIPROT
ORGANISM
PROTEIN VARIANTS
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
C161Y
mutation reported in ethylmalonic encephalopathy patients, drastic reduction in iron content
R163W
mutation reported in ethylmalonic encephalopathy patients
T136A
mutation reported in ethylmalonic encephalopathy patients
TEMPERATURE STABILITY
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
38
mutant C161Y, melting temperature
44
mutant T136A, melting temperature
47
mutant L55P, melting temperature
53
mutant R163W, melting temperature
64
wild-type, melting temperature
APPLICATION
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
medicine
-
the combined oral treatment with metronidazole and N-acetylcysteine is an effective therapy for ethylmalonic encephalopathy which is caused by mutations in the mitochondrial matrix sulfur dioxygenase ETHE1
REF.
AUTHORS
TITLE
JOURNAL
VOL.
PAGES
YEAR
ORGANISM (UNIPROT)
PUBMED ID
SOURCE
Viscomi, C.; Burlina, A.B.; Dweikat, I.; Savoiardo, M.; Lamperti, C.; Hildebrandt, T.; Tiranti, V.; Zeviani, M.
Combined treatment with oral metronidazole and N-acetylcysteine is effective in ethylmalonic encephalopathy
Nat. Med.
16
869-871
2010
Homo sapiens, Mus musculus
Manually annotated by BRENDA team
Kabil, O.; Motl, N.; Strack, M.; Seravalli, J.; Metzler-Nolte, N.; Banerjee, R.
Mechanism-based inhibition of human persulfide dioxygenase by gamma-glutamyl-homocysteinyl-glycine
J. Biol. Chem.
293
12429-12439
2018
Homo sapiens (O95571), Homo sapiens
Manually annotated by BRENDA team