Sulfide:quinone oxidoreductase, mitochondrial

Definition:

Catalyzes the oxidation of hydrogen sulfide with the help of a quinone, such as ubiquinone-10, giving rise to thiosulfate and ultimately to sulfane (molecular sulfur) atoms. Requires an additional electron acceptor; can use sulfite, sulfide or cyanide (in vitro). It is believed the in vivo electron acceptor is glutathione.

References:

[1]. Aaron P Landry, et al. Modulation of Catalytic Promiscuity during Hydrogen Sulfide Oxidation. ACS Chem Biol. 2018 Jun 15;13(6):1651-1658. [Content Brief]

[2]. Marouane Libiad, et al. Organization of the human mitochondrial hydrogen sulfide oxidation pathway. J Biol Chem. 2014 Nov 7;289(45):30901-10. [Content Brief]

[3]. Marisa W Friederich, et al. Pathogenic variants in SQOR encoding sulfide:quinone oxidoreductase are a potentially treatable cause of Leigh disease. J Inherit Metab Dis. 2020 Sep;43(5):1024-1036. [Content Brief]

[4]. Michael R Jackson, et al. Human sulfide:quinone oxidoreductase catalyzes the first step in hydrogen sulfide metabolism and produces a sulfane sulfur metabolite. Biochemistry. 2012 Aug 28;51(34):6804-15. [Content Brief]

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