What is Alpha-ketoglutarate dehydrogenase activated by?
α-Ketoglutarate dehydrogenase is regulated through the reversible glutathionylation of the enzyme’s cofactor lipoic acid. Evidence suggests that glutathionylation of KGDH represents an antioxidant response and is either enzyme catalysed or requires a unique microenvironment.
What is the inhibitor of α ketoglutarate dehydrogenase enzyme?
alpha-Keto-beta-methyl-n-valeric acid (KMV) inhibits KGDHC activity in living N2a cells in a dose- and time-dependent manner. Surprisingly, concentration of KMV that inhibit in situ KGDHC by 80% does not alter the mitochondrial membrane potential (MMP).
Is Alpha-ketoglutarate dehydrogenase a multi enzyme complex?
The α-KGDC is a multienzyme complex composed of three subunits (Fig. 1). The E1 subunit, encoded by the human OGDH gene, is a dehydrogenase that catalyzes the decarboxylation of α-KG, the first step required to produce succ-CoA.
What are the cofactors of alpha-ketoglutarate dehydrogenase?
Alpha-ketoglutarate dehydrogenase (α-KGDH) is a Krebs cycle enzyme, which catalyses the non-equilibrium reaction converting α-ketoglutarate, coenzyme A and NAD+ to succinyl-CoA, NADH and CO2, requiring thiamine pyrophosphate as a cofactor.
What happens when alpha-ketoglutarate?
Alpha-ketoglutarate (AKG) is a key molecule in the Krebs cycle determining the overall rate of the citric acid cycle of the organism. It is a nitrogen scavenger and a source of glutamate and glutamine that stimulates protein synthesis and inhibits protein degradation in muscles.
What is alpha-ketoglutarate dehydrogenase deficiency?
Alpha-ketoglutarate dehydrogenase deficiency is an autosomal recessive disorder caused by partial or total inactivation of the mitochondrial enzyme alpha-ketoglutarate dehydrogenase.
What does α-ketoglutarate dehydrogenase do?
Alpha-ketoglutarate dehydrogenase (α-KGDH) is a highly regulated enzyme, which could determine the metabolic flux through the Krebs cycle. It catalyses the conversion of α-ketoglutarate to succinyl-CoA and produces NADH directly providing electrons for the respiratory chain.
What does Alpha ketoglutarate dehydrogenase do?
What is the role of alpha-ketoglutarate?
What does Alpha-ketoglutarate dehydrogenase do?
What are the symptoms of succinate deficiency?
Succinic semialdehyde dehydrogenase deficiency is a disorder that can cause a variety of neurological problems. People with this condition typically have developmental delay, especially involving speech development; intellectual disability; and decreased muscle tone (hypotonia) soon after birth.
What cofactors does α-ketoglutarate dehydrogenase use?
What is the alpha ketoglutarate dehydrogenase complex?
The alpha-ketoglutarate dehydrogenase complex (KGDHC) is an important mitochondrial constituent, and deficiency of KGDHC is associated with a number of neurological disorders. KGDHC is composed of three proteins, each encoded on a different and well-characterized gene.
What co-factors are involved in oxidative decarboxylation of ketoglutarate?
Oxidative decarboxylation of both a-ketoglutarate and pyruvate dehydrogenases, share the co-factors listed above. The oxidative decarboxylation of α-ketoglutarate is catalysed by an enzyme complex that is structurally similar to the pyruvate dehydrogenase complex.
Can alpha-ketoglutarate dehydrogenase regulate metabolic flux through the Krebs cycle?
Alpha-ketoglutarate dehydrogenase (α-KGDH) is a highly regulated enzyme, which could determine the metabolic flux through the Krebs cycle.
What is branched chain alpha keto acid dehydrogenase?
Branched-Chain Alpha-Keto Acid Dehydrogenase Complex. Branched-chain α-keto acid dehydrogenase complex (BCKDC) is a mitochondrial multienzyme complex that catalyzes a series of reactions that form the first irreversible step in the catabolism of the essential branched-chain amino acids: leucine, isoleucine, and valine.