The role of glutamate oxaloacetate transaminases in sulfite biosynthesis and H₂S metabolism.

ABSTRACT: Molybdenum cofactor deficiency and isolated sulfite oxidase deficiency are two rare genetic disorders that are caused by impairment of the mitochondrial enzyme sulfite oxidase. Sulfite oxidase is catalyzing the terminal reaction of cellular cysteine catabolism, the oxidation of sulfite to sulfate. Absence of sulfite oxidase leads to the accumulation of sulfite, which has been identified as a cellular toxin. However, the molecular pathways leading to the production of sulfite are still not completely understood. In order to identify novel treatment options for both disorders, the understanding of cellular cysteine catabolism - and its alterations upon loss of sulfite oxidase - is of utmost importance. Here we applied a new detection method of sulfite in cellular extracts to dissect the cont

SUBMITTER: Mellis AT 

PROVIDER: S-EPMC7711302 | biostudies-literature | 2021 Jan

REPOSITORIES: biostudies-literature