Persulfide sulfur is dioxygenated

Stable Identifier
R-HSA-1614605
Type
Reaction [transition]
Species
Homo sapiens
Compartment
mitochondrial inner membrane, mitochondrial matrix
ReviewStatus
5/5
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Persulfide sulfur is dioxygenated
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The sulfur dioxygenase ETHE1 converts persulfides to sulfite. Loss of this activity leads to the rare ethylmalonyl encephalopathy where the body can no longer detoxify H2S (Tiranti et al, 2009).
Literature References
PubMed ID Title Journal Year
19136963 Loss of ETHE1, a mitochondrial dioxygenase, causes fatal sulfide toxicity in ethylmalonic encephalopathy

Tiranti, V, Viscomi, C, Hildebrandt, T, Di Meo, I, Mineri, R, Tiveron, C, Levitt, MD, Prelle, A, Fagiolari, G, Rimoldi, M, Zeviani, M

Nat Med 2009
Participants
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Output
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as an event of
Catalyst Activity

sulfur dioxygenase activity of ETHE1:2Zn2+ [mitochondrial matrix]

Physical Entity
ETHE1:2Zn2+ [mitochondrial matrix] (Homo sapiens)
Activity
sulfur dioxygenase activity (GO:0050313)
Orthologous Events
Persulfide sulfur is dioxygenated (Bos taurus)
Persulfide sulfur is dioxygenated (Caenorhabditis elegans)
Persulfide sulfur is dioxygenated (Canis familiaris)
Persulfide sulfur is dioxygenated (Danio rerio)
Persulfide sulfur is dioxygenated (Drosophila melanogaster)
Persulfide sulfur is dioxygenated (Mus musculus)
Persulfide sulfur is dioxygenated (Rattus norvegicus)
Persulfide sulfur is dioxygenated (Sus scrofa)
Persulfide sulfur is dioxygenated (Xenopus tropicalis)
Authored
Stephan, R  (2010-10-24)
Reviewed
D'Eustachio, P  (2011-10-13)
Created
Jassal, B  (2011-09-29)
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