Name=2-oxoglutarate dehydrogenase E1 component (α-Ketoglutarate dehydrogenase)
HGNCid = 8124
Symbol = OGDH
Names= Alpha-ketoglutarate dehydrogenase
Chromosome = 7
Arm = p
Band = 13
LocusSupplementaryData = -p11.2
Functions=converts Alpha ketoglutarate to
Transketolasecentral region, DehydrogenaseE1 component
Catalytic_activity=2-oxoglutarate + [dihydrolipoyllysine-residue succinyltransferase] lipoyllysine = [dihydrolipoyllysine-residue succinyltransferase] S-succinyldihydrolipoyllysine + CO(2)
Thiamine pyrophosphate lipoate, FAD, NAD, CoA
Diseases=Alpha-Ketoglutarate Dehydrogenase Deficiency
Homo sapiens"; homologs: ubiquitousamong oxidatively respiring organisms
Cells= ubiquitous among aerobic cells
mitochondrion, mitochondrial matrix
Citric acid cycle, Lysine degradation, Tryptophan metabolism
ECnumber = 18.104.22.168
Codes= EntrezGene|4967; OMIM|203740; RefSeq|NM_001003941; UniProt|Q02218
Oxoglutarate dehydrogenase (aka α-ketoglutarate dehydrogenase) is an enzyme complex most commonly known for its role in the
citric acid cycle.
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-oxoglutarateand a third specific branched-chain α-keto acids.
Nomenclature and classification
The official name of this enzyme is oxoglutarate dehydrogenase (OGDC) although it is also commonly known as α-ketoglutarate dehydrogenase (AKGDH).
This enzyme participates in three different pathways:
Citric acid cycle(KEGG link: [http://www.genome.jp/kegg/pathway/map/map00720.html MAP00020] )
Lysinedegradation (KEGG link: [http://www.genome.jp/kegg/pathway/map/map00310.html MAP00310] )
Tryptophanmetabolism (KEGG link: [http://www.genome.jp/kegg/pathway/map/map00380.html MAP00380] )
The following values are from "
Azotobacter vinelandii" (1):
*KM: 0.14 ± 0.04 mM
*Vmax : 9 ± 3 μmol.min-1.mg-1
Citric acid cycle
This reaction proceeds in three steps:
reductionof NAD+ to NADH,
* and subsequent transfer to CoA, which forms the end product,
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
Oxoglutarate dehydrogenase is a key control point in the citric acid cycle. It is inhibited by its products,
succinyl CoAand NADH. A high energy charge in the cell will also be inhibitive.
2-Oxo-glutarate dehydrogrenase is an
autoantigenrecognized in primary biliary cirrhosis, a form of acute liver failure. These antibodiesappear to recognize oxidized proteinthat has resulted from inflammatory immune responses. Some ofthese inflammatory responses are explained by gluten sensitivity.cite journal | author = Leung PS, Rossaro L, Davis PA, "et al" | title = Antimitochondrial antibodies in acute liver failure: Implications for primary biliary cirrhosis | journal = Hepatology| volume = 46| issue = | pages = 1436| year = 2007 | pmid = 17657817 | doi = 10.1002/hep.21828] Other mitochondrial autoantigensinclude pyruvate dehydrogenaseand branched-chain alpha-keto acid dehydrogenase complex, which are antigens recognized by anti-mitochondrial antibodies.
# Bunik V, Westphal AH, de Kok A: "Kinetic properties of the 2-oxoglutarate dehydrogenase complex from Azotobacter vinelandii evidence for the formation of a precatalytic complex with 2-oxoglutarate." Eur J Biochem 2000; 267(12): 3583-91. PMID 10848975.
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