SUOX

Sulfite oxidase is a mitochondrial enzyme that catalyzes the oxidation of sulfite to sulfate, representing the final step in the degradation of sulfur-containing amino acids cysteine and methionine. Biallelic mutations cause sulfite oxidase deficiency, an autosomal recessive disorder characterized by severe neurological abnormalities that are often fatal in early childhood. The gene shows tolerance to loss-of-function variants (pLI near zero), consistent with its recessive inheritance pattern.