Persulfide dioxygenase ETHE1, mitochondrial (ETHE1)

Sulfur dioxygenase that plays an essential role in hydrogen sulfide catabolism in the mitochondrial matrix. Hydrogen sulfide (H(2)S) is first oxidized by SQRDL, giving rise to cysteine persulfide residues.

ETHE1 absorbs molecular oxygen to catalyze the oxidation of the persulfide, once it's been transferred to a thiophilic acceptor, such as glutathione (R-SSH). Plays an important role in metabolic homeostasis in mitochondria by metabolizing hydrogen sulfide and preventing the accumulation of supraphysiological H(2)S levels that have toxic effects, because of the inhibition of cytochrome c oxidase.