Reactome | Persulfide sulfur is dioxygenated

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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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 Levitt, MD, Zeviani, M, Rimoldi, M, Fagiolari, G, Di Meo, I, Tiranti, V, Prelle, A, Mineri, R, Viscomi, C, Tiveron, C, Hildebrandt, T	Nat Med	2009