11. Others may have mutations in certain subunits of succinate dehydrogenase, a mitochondrial enzyme. 12. Purple dashed arrow shows diffusion of succinate to block PHD ( Pathway 2 ). 13. Therefore, increasing ?-ketoglutarate levels can offset the effect of succinate accumulation. 14. Alternative approaches have altered the redox and ATP balance to optimize the succinate yield. 15. Succinate semialdehyde is considered a reactive carbonyl and may lead to increased oxidative stress.16. SDHA contains the FAD binding site where succinate is deprotonated and converted to fumarate. 17. During catalysis, isocitrate is aldol cleavage results in the release of succinate and glyoxylate. 18. It is an inhibitor of succinate dehydrogenase. 19. COO is lost in each step and succinate ( a four carbon compound ) is produced. 20. Because the mutated SDH enzyme cannot convert succinate to fumarate, succinate accumulates in the cell.
