Sulfite-dependent regulation of mitochondrial function by sulfite oxidase (B08)

• Subject Area: Biochemistry
• Term: since 2016
• Project identifier: Deutsche Forschungsgemeinschaft (DFG) - Project number 269925409

Mitochondrial sulfite oxidase (SOX) is the most important molybdenum-dependent enzyme in humans. It catalyses the final step in cysteine catabolism within the intermembrane space of mitochondria. A deficiency in SOX is associated with rapidly progressing neurodegeneration resulting in most cases in early childhood death. Our mouse model developed for SOX deficiency (SOXD) disclosed an unexpected accumulation of H2S as novel hallmark of the disease. In the third funding period we aim to understand the mechanistic basis how H2S and altered persulfidation affecting mitochondria in different tissues of Suox–/– mice.

• DFG Programme: Collaborative Research Centres
• Subproject of: SFB 1218: Mitochondrial regulation of cellular function
• Applicant Institution: Universität zu Köln
• Project Head: Professor Dr. Günter Schwarz