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Oxoglutarate dehydrogenase
Oxoglutarate dehydrogenase (aka α-ketoglutarate dehydrogenase) is an enzyme complex most commonly known for its role in the citric acid cycle. Additional recommended knowledge
UnitsMuch like pyruvate dehydrogenase complex, this enzyme forms a complex composed of three components:
In fact, three classes of these multienzyme complexes have been characterized, one specific for pyruvate, a second specific for 2-oxoglutarate and a third specific branched-chain α-keto acids. PropertiesNomenclature and classificationThe official name of this enzyme is oxoglutarate dehydrogenase (OGDC) although it is also commonly known as α-ketoglutarate dehydrogenase (AKGDH).
Metabolic pathwaysThis enzyme participates in three different pathways:
Kinetic propertiesThe following values are from Azotobacter vinelandii (1):
Citric acid cycleReactionThe reaction catalyzed by this enzyme in the citric acid cycle is:
This reaction proceeds in three steps:
ΔG°' for this reaction is -7.2 kcal mol-1. The energy needed for this oxidation is conserved in the formation of a thioester bond of succinyl CoA. RegulationOxoglutarate dehydrogenase is a key control point in the citric acid cycle. It is inhibited by its products, succinyl CoA and NADH. A high energy charge in the cell will also be inhibitive.
Pathology2-Oxo-glutarate dehydrogrenase is an autoantigen recognized in primary biliary cirrhosis, a form of acute liver failure. These antibodies appear to recognize oxidized protein that has resulted from inflamatory immune responses. Some of these inflamatory responses are explained by gluten sensitivity.[1] Other mitochondrial autoantigens include pyruvate dehydrogenase and branched-chain alpha-keto acid dehydrogenase complex, which are antigens recognized by anti-mitochondrial antibodies. References
Categories: EC 1.2.4 | Autoantigens |
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This article is licensed under the GNU Free Documentation License. It uses material from the Wikipedia article "Oxoglutarate_dehydrogenase". A list of authors is available in Wikipedia. |