What happens to ketoglutarate?
In reaction 4 of the citric acid cycle, alpha-ketoglutarate loses a carbon dioxide molecule and coenzyme A is added in its place. The decarboxylation occurs with the help of NAD, which is converted to NADH. The resulting molecule is called succinyl-CoA.
What happens if succinate dehydrogenase is inhibited?
A complete lack of succinate dehydrogenase activity will hamper electron flow to both respiratory chain complex III and the quinone pool, resulting in a major oxidative stress known to promote tumor formation in human.
What do you need to know about a-ketoglutarate dehydrogenase?
alpha-ketoglutarate dehydrogenase. (redirected from a-ketoglutarate dehydrogenase) an enzyme that catalyzes the oxidative decarboxylation of 2-ketoglutaric acid to succinyldihydrolipoate; the succinyl group is later transferred to CoA and the reduced lipoate is oxidized by NAD +; a complex that is a part of the tricarboxylic acid cycle.
What kind of dehydrogenase is α-kgdh?
α-KGDH is a complex enzyme consisting of multiple copies of three subunits (see figure 2), a thiamine pyrophosphate-dependent dehydrogenase (E1), dihydrolipoamide succinyltransferase (E2) and dihydrolipoamide dehydrogenase (E3), the latter being a flavoprotein (Koike et al. 1974; Koike & Koike 1976).
Which is the end product of Oxoglutarate dehydrogenase?
α-ketoglutarate + NAD + + CoA → Succinyl CoA + CO 2 + NADH. Oxoglutarate dehydrogenase (α-Ketoglutarate dehydrogenase) This reaction proceeds in three steps: decarboxylation of α-ketoglutarate, reduction of NAD + to NADH, and subsequent transfer to CoA, which forms the end product, succinyl CoA. ΔG°’ for this reaction is -7.2 kcal mol −1.
What are the effects of alpha ketoglutarate deficiency?
A rare, genetic, inborn error of metabolism disorder characterized by neonatal-onset of developmental delay, hypotonia, hepatomegaly, lactic acidemia, increased creatine kinase levels, elevated alpha-ketoglutaric acid in urine, and a decreased plasma beta-hydroxybutyrate-to-acetoacetate ratio.