Article

Sulfite oxidase deficiency causes persulfidation loss and H <sub>2</sub> S release

2024-03-13

Abstract excerpt

Sulfite 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 raise in downstream products S -sulfocysteine (SSC), mediating excitotoxicity, and thiosulfate, a catabolic intermediate/product of H 2 S metabolism. Here, we report a full-body knock-out mouse model for SOX deficiency (SOXD) with a sev...

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Literature Corpus work: 57c7a39c-d9e5-5094-82d6-dae21ee42447
DOI: 10.1101/2024.03.13.584820

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