Persulfide sulfur is dioxygenated

Stable Identifier
R-HSA-1614605
Type
Reaction [transition]
Species
Homo sapiens
Compartment
Locations in the PathwayBrowser
General
SVG |   | PPTX  | SBGN
Click the image above or here to open this reaction in the Pathway Browser
The layout of this reaction may differ from that in the pathway view due to the constraints in pathway layout

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
Participant Of
Catalyst Activity
Catalyst Activity
Title
sulfur dioxygenase activity of ETHE1:2Zn2+ [mitochondrial matrix]
Physical Entity
Activity
Orthologous Events
Authored
Reviewed
Created