Succinic dehydrogenase subunit A deficiency (OMIM #252011) Succinic dehydrogenase subunit A (SDHA), a flavoprotein with FAD as cofactor, is a nuclear encoded and catalytic subunit of Complex II (succinate dehydrogenase) which oxidizes succinate to fumarate in the Krebs cycle and transfers electrons to ubiquinone in the mitochondrial electron transport chain [115]
-dicarboxyethyl) cysteine, 3- (carboxymethoxy) -alanine, S-carboxymethyl-glutathione (glutaconic acid), S-carboxymethyl-cysteinylglycine, (S-carboxymethyl) -lysyl-cysteine, S-dicarboxymethyl-glutathione, S-carboxymethyl-cysteine, S- (1, 2-dicarboxyethyl) glutathione and S- (1, 2-dicarboxyethyl) -cysteine, -glutamyl-L-cysteinylglycine or -glutamyl-cysteinylglycine, luminal essence, lumetamide, lobemide, rubescenmide, zanthosine, zanthosinamide, dioxamide, zanthoamine, and zanthomamide
To explore the role of the side chain, a group of three monophenols namely, isoeugenol, dihydroeugenol, and eugenol, differing by one double bond or its position in the chain, was also examined
Entirely different mechanisms
Clinical improvement was also observed in oxidative stress markers, with improvements in methionine levels (p=0.05), homocysteine levels (SAH, p=0.007) and the ratio of S-adenosylmethionine (SAMe) to SAH (p=0.007), indicating improvement with methylation at the cellular level and a decrease in oxidative stress in the body 2
