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001048140 1001_ $$0P:(DE-HGF)0$$aFu, Chun-Yu$$b0
001048140 245__ $$aSulfite oxidase deficiency causes persulfidation loss and hydrogen sulfide release
001048140 260__ $$aAnn Arbor, Mich.$$bASCJ$$c2025
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001048140 520__ $$aSulfite oxidase (SOX) deficiency is a rare inborn error of cysteine metabolism resulting in severe neurological damage. In patients, sulfite accumulates to toxic levels, causing a rise in the downstream products S-sulfocysteine, which mediates excitotoxicity, and thiosulfate, a catabolic intermediate/product of hydrogen sulfide (H2S) metabolism. Here, we report a full-body knockout mouse model for SOX deficiency (SOXD) with a severely impaired phenotype. Among the urinary biomarkers, thiosulfate showed a 45-fold accumulation in SOXD mice, representing the major excreted S-metabolite. Consistently, we found increased plasma H2S, which was derived from sulfite-induced release from persulfides, as demonstrated in vitro and in vivo. Mass spectrometry analysis of total protein persulfidome identified a major loss of S-persulfidation in 20% of the proteome, affecting enzymes in amino acids, fatty acid metabolism, and cytosolic iron-sulfur cluster biogenesis. Urinary amino acid profiles indicated metabolic rewiring and mitochondrial dysfunction, thus identifying an altered H2S metabolism and persulfidation in SOXD. Finally, oxidized glutathione and glutathione trisulfide were able to scavenge sulfite in vitro and in vivo, extending the lifespan of SOXD mice and providing a mechanistic concept of sulfite scavenging for the treatment of this severe metabolic disorder of cysteine catabolism.
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001048140 7001_ $$0P:(DE-HGF)0$$aKohl, Joshua B.$$b1
001048140 7001_ $$0P:(DE-HGF)0$$aLiebsch, Filip$$b2
001048140 7001_ $$0P:(DE-HGF)0$$aD’Andrea, Davide$$b3
001048140 7001_ $$0P:(DE-HGF)0$$aDitrói, Tamás$$b4
001048140 7001_ $$0P:(DE-HGF)0$$aOgata, Seiryo$$b5
001048140 7001_ $$0P:(DE-HGF)0$$aNeuser, Franziska$$b6
001048140 7001_ $$0P:(DE-HGF)0$$aMai, Max$$b7
001048140 7001_ $$0P:(DE-HGF)0$$aMellis, Anna T.$$b8
001048140 7001_ $$0P:(DE-HGF)0$$aKouroussis, Emilia$$b9
001048140 7001_ $$0P:(DE-HGF)0$$aMorita, Masanobu$$b10
001048140 7001_ $$0P:(DE-HGF)0$$aGehling, Titus$$b11
001048140 7001_ $$0P:(DE-HGF)0$$aSantamaria-Araujo, José Angel$$b12
001048140 7001_ $$0P:(DE-HGF)0$$aYeo, Sin Yuin$$b13
001048140 7001_ $$0P:(DE-Juel1)180330$$aEndepols, Heike$$b14$$ufzj
001048140 7001_ $$0P:(DE-HGF)0$$aKřížková, Michaela$$b15
001048140 7001_ $$0P:(DE-HGF)0$$aKozich, Viktor$$b16
001048140 7001_ $$0P:(DE-HGF)0$$aKrueger, Marcus$$b17
001048140 7001_ $$0P:(DE-HGF)0$$aHennermann, Julia B.$$b18
001048140 7001_ $$0P:(DE-HGF)0$$aBarayeu, Uladzimir$$b19
001048140 7001_ $$00000-0002-0623-1710$$aAkaike, Takaaki$$b20
001048140 7001_ $$0P:(DE-HGF)0$$aNagy, Peter$$b21
001048140 7001_ $$0P:(DE-HGF)0$$aFilipovic, Milos$$b22
001048140 7001_ $$0P:(DE-HGF)0$$aSchwarz, Guenter$$b23$$eCorresponding author
001048140 773__ $$0PERI:(DE-600)2018375-6$$a10.1172/JCI181299$$gVol. 135, no. 21, p. e181299$$n21$$pe181299$$tThe journal of clinical investigation$$v135$$x0021-9738$$y2025
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