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Topic: Alpha-ketoglutarate

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oxoglutarate dehydrogenase EC 1.2.4.2 and EC 1.2.1.52; component of a multi-enzyme complex which decarboxylates alpha ketoglutarate to form succinyl coA in the Krebs' cycle. www.crisp.cit.nih.gov /Thesaurus/00005945.htm

alpha-Ketoglutarate Dehydrogenase E1 Backpage Protein: 2-oxoglutarate dehydrogenase E1 component, mitochondrial precursor (EC 1.2.4.2) (Alpha-ketoglutarate dehydrogenase). Definition: Homo sapiens oxoglutarate (alpha-ketoglutarate) dehydrogenase (lipoamide) (OGDH), mRNA. IT CONTAINS MULTIPLE COPIES OF 3 ENZYMATIC COMPONENTS: 2-OXOGLUTARATE DEHYDROGENASE (E1), DIHYDROLIPOAMIDE SUCCINYLTRANSFERASE (E2) AND LIPOAMIDE DEHYDROGENASE (E3). www.genmapp.org /MAPPSet-Human/GenMAPP.org_MAPPs/Metabolic_MAPPs/_Support/Hs_Krebs-TCA_Cycle/alpha-Ketoglutarate_Dehydrogenase_E1_Q02218.htm

Ketoglutarate Dehydrogenase Complex - Unified Search Environment component of a multi-enzyme complex which decarboxylates alpha ketoglutarate to form succinyl coA in the Krebs' cycle. www.use.hcn.com.au /subject.%60Ketoglutarate%20Dehydrogenase%20Complex%60/home.html

BIOCHEMISTRY I - CITRIC ACID CYCLE Oxidative decarboxylation of alpha -ketoglutarate to succinyl CoA catalyzed by Oxidation of isocitrate to alpha -ketoglutarate catalyzed by Pyruvate dehydrogenase is a large, integrated array of enzymes. www.sbuniv.edu /~ggray/CHE3364/b1c20out.html

Catabolism of alpha -Ketoglutarate by a sucA Mutant of Bradyrhizobium japonicum: Evidence for an Alternative Tricarboxylic Acid Cycle -- Green et al. 182 (10): 2838 -- The Journal of Bacteriology Catabolism of alpha -Ketoglutarate by a sucA Mutant of Bradyrhizobium japonicum: Evidence for an Alternative Tricarboxylic Acid Cycle -- Green et al. -ketoglutarate decarboxylase; SSDH, succinic semialdehyde dehydrogenase; SSA, succinic semialdehyde; GAT, Succinic semialdehyde dehydrogenase was found in all of the B. jb.asm.org /cgi/content/full/182/10/2838

body Glutamate dehydrogenase is a homohexameric member of the amino acid dehydrogenase superfamily of enzymes that catalyzes the NAD(P)+-linked oxidative deamination of L-amino acids to their corresponding alpha -keto acids. Recent studies have utilized commercially available glutamate dehydrogenase from beef liver to look at binding sites on glutamate dehydrogenase, as well as the portion of the coenzyme responsible for interaction with glutamate dehydrogenase (4, 8). Mammalian glutamate dehydrogenase, on the other hand, is able to utilize both coenzymes with equal efficacy. www.arches.uga.edu /~cwildman/bcmb8010/body.html

Declines in mitochondrial respiration during cardiac reperfusion: age-dependent inactivation of alpha-ketoglutarate dehydrogenase. Thus, our results suggest a plausible mechanism for age-dependent, reperfusion-induced declines in mitochondrial function and identify alpha-ketoglutarate dehydrogenase as a likely site of free radical-mediated damage. Declines in mitochondrial respiration during cardiac reperfusion: age-dependent inactivation of alpha-ketoglutarate dehydrogenase. To gain insight into potential sites of oxidative damage, the present study was undertaken to identify specific mitochondrial protein(s) inactivated during ischemia and reperfusion and to determine which of these losses in activity are responsible for observed declines in mitochondrial respiration. www.arclab.org /medlineupdates/abstract_10359773.html

ORPHANET - Rare diseases - Orphan drugs Oxoglutaric aciduria is an autosomal recessive condition due to alpha ketoglutarate dehydrogenase deficiency, an enzyme involved in the trasformation of alpha ketoglutarate into succinyl-CoA in the Krebs cycle. However the enzyme should be measured for confirmation, as alpha ketoglutaric aciduria is a very common, non-specific finding. The structure of the enzyme is a complex one: it comprises 3 subunits, among which the E3 subunit, common to pyruvate dehydrogenase and branched aminoacids decarboxylation (?). orphanet.infobiogen.fr /consor/cgi-bin/OC_Exp.php?Lng=GB&Expert=31

BC2001Glycolysis&TheTCA:The_Citric_Acid_Cycle the next step is an oxidative decarboxylation of isocitrate to alpha-ketoglutarate in an NAD+ dependent reaction catalysed by NAD+ dependent isocitrate dehydrogenase. In the first step the asp carboxylate removes a proton from the alpha carbon of acetyl-CoA, the electrons go to form a double bond between the two carbon atoms, and the pi electrons from the C-O double bond pick up a proton off of the N3 atom of a His imidazole ring. Alpha-KG dehydrogenase is inhibited by succinyl-CoA and NADH aci.mta.ca /Courses/Biology/BioWeb/2001TCAcycle.html

Alpha ketoglutarate dehydrogenase Alpha ketoglutarate dehydrogenase serves to convert NADH + succinyl-CoA + CO2 into alpha-ketoglutarate + CoA + NAD+. This is an enzyme that acts in the alpha-ketoglutarate pathway, an intermediate pathway in the Krebs cycle in which amino acid derivatives enter the cycle. www.cartage.org.lb /en/themes/Reference/dictionary/Biologie/A/375.html

Functional analysis in Saccharomyces cerevisiae of naturally occurring amino acid substitutions in human dihydrolipoamide dehydrogenase -- Lanterman et al. 5 (10): 1643 -- Human Molecular Genetics To address this issue, we asked whether the human enzyme could restore function to the [alpha] -ketoacid dehydrogenase complexes in a yeast strain deficient in endogenous dihydrolipoamide dehydrogenase. Regions within dihydrolipoamide dehydrogenase involved in association with other proteins of the complexes are poorly defined, and despite high amino acid sequence conservation through evolution, it is unknown if dihydrolipoamide dehydrogenases are functionally equivalent across species. Human dihydrolipoamide dehydrogenases with selected amino acid substitutions were then tested in the null strain for their ability to restore function. hmg.oupjournals.org /cgi/content/full/5/10/1643

Le Magazine, April 2000 - Abstracts: Coq10 And Adhd The results are consistent with the conclusion that the ubiquinone pool undergoes an oxidative modification during lipid peroxidation, to a form that can no longer function as a component of the respiratory chain. Lipid peroxidation also led to a partial inhibition of the succinate dehydrogenase and cytochrome c oxidase activities and a minor decrease of the cytochrome c and cytochrome a contents. Reduction of endogenous ubiquinone prevented lipid peroxidation as well as the concomitant modification of ubiquinone and inactivation of the respiratory chain. www.lef.org /magazine/mag2000/april00-abs.html

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. www.arclab.org /medlineupdates/abstract_10672230.html

Alpha Keto Acid Dehydrogenase Complexes (1st) by M. S. Patel , Mulchand S. Patel (Other Contributor) ISBN: 3764351810 Alpha Keto Acid Dehydrogenase Complexes (1st) by M. Patel, Mulchand S. Patel (Other Contributor) ISBN: 3764351810 Found in all organisms, the alpha-keto acid dehydrogenase complexes have central roles in cellular metabolism and are major sites of regulation. A variety of experimental approaches together with results presented in this book should serve as a resource for beginning to established investigators in thefield, as well as scientists who are interested in mitochondria, dehydrogenases, kinases, phosphatases, lipoic acid, thiamine pyrophosphate, and enzyme complexes. www.campusi.com /isbn_3764351810.htm

Abstract KEY WORDS: alpha -ketoglutarate dehydrogenase, alpha -ketoglutarate, alpha -ketoadipate, inactivation during catalysis. The activity of some muscle alpha -ketoglutarate dehydrogenase complexes (KGDC) decreases during their enzymatic reaction as a result of inactivation of the first component of the complex, namely, alpha -ketoglutarate dehydrogenase (KGD). Submitted December 25, 1996; revision submitted June 5, 1997. www.protein.bio.msu.su /biokhimiya/contents/v62/abs/62091137.htm

Alpha-ketoglutarate dehydrogenase deficiency You have come across our Alpha-ketoglutarate dehydrogenase deficiency article. Unfortunately, work on Alpha-ketoglutarate dehydrogenase deficiency has not finished and we are still working on the contents. Please come back to this page in the future when more definitive content is published on Alpha-ketoglutarate dehydrogenase deficiency. www.bambooweb.com /articles/a/l/Alpha-ketoglutarate_dehydrogenase_deficiency.html

Alterations in mitochondrial function in a mouse model of hypertrophic cardiomyopathy -- Lucas et al. 284 (2): 575 -- AJP - Heart and Circulatory Physiology Selective inactivation of alpha-ketoglutarate dehydrogenase and pyruvate dehydrogenase: reaction of lipoic acid with 4-hydroxy-2- nonenal. Declines in mitochondrial respiration during cardiac reperfusion: age-dependent inactivation of alpha-ketoglutarate dehydrogenase. Dehydrogenase activities and NADH-linked electron transport chain components ajpheart.physiology.org /cgi/content/full/284/2/H575

Lipoic Acid Pyruvate dehydrogenase and alpha-ketoglutarate dehydrogenase reduced dehydroascorbic acid to ascorbic acid in an alpha-lipoic acid, coenzyme A, and pyruvate or alpha-ketoglutarate dependent fashion. Thus, mechanisms of reduction of alpha-lipoate are highly tissue-specific and effects of exogenously supplied alpha-lipoate are determined by tissue glutathione reductase and dihydrolipoamide dehydrogenase activity. The experiment has shown that a complex of functionally related vitamins including thiamine, lipoate, D-pantothenate, nicotinate and riboflavine in "pyruvate-dehydrogenase" ratios decreases inhibition of the activity of alpha-keto acid dehydrogenases in the brain and liver with thiopental anesthesia, intensifies arrival of [35S]-lipoate to the brain and decreases acute toxicity of sodium thiopental (TnNa). www.cfsn.com /lipoic.html

OUTPUT from blastx for 5009-0-82-A06 (NM_002541) oxoglutarate (alpha-ketoglutarate) dehydrogenase (lipoamide); oxoglutarate dehydrogenase (lipoamide) [Homo sapiens] spQ02218ODO1_HUMAN 2-oxoglutarate dehydrogenase E1 component, mitochondrial precursor (Alpha-ketoglutarate dehydrogenase) dbjBAA01393.1 225 3e-58 pirT50644 oxoglutarate dehydrogenase (lipoamide) (EC 1.2.... (NM_115399) 2-oxoglutarate dehydrogenase, E1 subunit - like protein [Arabidopsis thaliana] pirT47680 probable oxoglutarate dehydrogenase (lipoamide) (EC 1.2.4.2) E1 chain - Arabidopsis thaliana embCAB75899.1 www.ca.embnet.org /reith/tetra/tetra5/5009-0-82-A06.html

Report.2002-05 GO:0008751, NAD(P)H2 dehydrogenase: was a redundant grouping term with only one child. www.geneontology.org /MonthlyReports/Report.2002-05

Eric Knapp Inactivation of alpha-Ketoglutarate Dehydrogenase during Its Enzymatic Reaction The nitration product 5-nitro-gamma-tocopherol is increased in the Alzheimer brain. wheat.pw.usda.gov /~vcarollo/SIB/2002/website/tca/knapp/knapp.html

Search Results alpha-ketoglutarate dehydrogenase (2-oxoglutarate dehydrogenase; pyruvate dehydrogenase component E1) It contains multiple copies of 3 enzymatic components: alpha-ketoglutarate dehydrogenase E1 (or 2-oxoglutarate dehydrogenase), dihydrolipoamide succinyltransferase (E2) and lipoamide dehydrogenase (E3). Hits in gapped BLAST to alpha-ketoglutarate dehydrogenase sequences, e.g. www.stdgen.lanl.gov /cgi-bin/gene_id_search.cgi?dbname=chlamy&gene_id=CT054

Bradyrhizobium japonicum does not require alpha-ketoglutarate dehydrogenase for growth on succinate or malate -- Green and Emerich 179 (1): 194 -- The Journal of Bacteriology Green, L. S., Li, Y., Emerich, D. W., Bergersen, F. J., Day, D. Catabolism of alpha -Ketoglutarate by a sucA Mutant of Bradyrhizobium japonicum: Evidence for an Alternative Tricarboxylic Acid Cycle. Bradyrhizobium japonicum does not require alpha-ketoglutarate dehydrogenase for growth on succinate or malate -- Green and Emerich 179 (1): 194 -- The Journal of Bacteriology dehydrogenase, was cloned from Bradyrhizobium japonicum USDA110, and its intl-jb.asm.org /cgi/content/abstract/179/1/194

changpub Artificial cells microencapsulated multienzyme system for converting urea and ammonia to amino acid using alpha- ketoglutarate and glucose as substrate. Effects of glucose dehydrogenase in converting urea and ammonia into amino acid using artificial cells. "Immobilized and kinetics of lactate dehydrogenase at a rotating nylon disk: Biotechnology and Bioengineering, 32: 213-219. www.medicine.mcgill.ca /artcell/changpub.htm

Zn2+ Inhibits alpha -Ketoglutarate-stimulated Mitochondrial Respiration and the Isolated alpha -Ketoglutarate Dehydrogenase Complex -- Brown et al. 275 (18): 13441 -- Journal of Biological Chemistry Zn2+ Inhibits alpha -Ketoglutarate-stimulated Mitochondrial Respiration and the Isolated alpha -Ketoglutarate Dehydrogenase Complex -- Brown et al. dehydrogenase); GDH, glutamate dehydrogenase; MDH, malate dehydrogenase; LipS ), malate dehydrogenase was unaffected, and succinate dehydrogenase www.jbc.org /cgi/content/full/275/18/13441

02C/D/E: Thiamine Transporter, Thiamine Pyrophosphokinase, and alpha-Ketoglutarate Dehydrogenase: Thiamine-Responsive Megaloblastic Anemia Thiamine-responsive megaloblastic anemia (TRMA, see OMIM 249270) can be caused by defects in a putative thiamine transporter, thiamine pyrophosphokinase (TPK), and a-ketoglutarate dehydrogenase (KGDH). The putative thiamine transporter, SLC19A2, is homologous to reduced folate carrier proteins, and may bring thiamine into cells. KGDH is one of the thiamine-dependent dehydrogenases that bind TPP by an E1 carboxylase (see also section 2A and section 2B). www.kmmutants.org /02CDE.php

Inactivation of the 2-oxo acid dehydrogenase complexes upon generation of intrinsic radical species -- Bunik and Sievers 269 (20): 5004 -- European Journal of Biochemistry incubation of MNP with the pyruvate or 2-oxoglutarate dehydrogenase   Inactivation of 2-oxoglutarate dehydrogenase from pig heart upon incubation with its substrates and/or products (Reaction conditions are indicated in Materials and methods). dehydrogenase (E3) are organized in highly ordered structures www.ejbiochem.org /cgi/content/full/269/20/5004

Characterization of Two cDNAs Encoding Mitochondrial Lipoamide Dehydrogenase from Arabidopsis -- Lutziger and Oliver 127 (2): 615 -- PLANT PHYSIOLOGY Arabidopsis has two nuclear-encoded mitochondrial lipoamide dehydrogenase genes. All these multienzyme complexes are found in the mitochondria with pyruvate dehydrogenase also occurring in plastids. dehydrogenase), E2 (the dihyrdolipoyl transacylase), and E3 (the www.plantphysiol.org /cgi/content/full/127/2/615

Diabetes: Impaired Fuel-Stimulated Insulin Secretion in a MODY-3 Model Is Accompanied by Decreased Alpha-Ketoglutarate Dehydrogenase Activity Diabetes: Impaired Fuel-Stimulated Insulin Secretion in a MODY-3 Model Is Accompanied by Decreased Alpha-Ketoglutarate Dehydrogenase Activity mRNA levels of the E1 [Alpha] subunit of [Alpha]-ketoglutarate dehydrogenase ([Alpha]KGDH) is decreased by 50% in P291C expressing cells compared to the non-induced controls. Northern analysis and TCA cycle enzyme activity assays identified [Alpha]-ketoglutarate dehydrogenase ([Alpha]KGDH) as a potential target of P291C overexpression. findarticles.com /cf_dls/m0922/5_49/62891482/p1/article.jhtml

GENERATION OF REACTIVE OXYGEN SPECIES BY BRAIN MITOCHONDRIA MEDIATED BY ALPHA-KETOGLUTARATE DEHYDROGENASE Rates of H2O2 production with respiratory substrates malate, pyruvate, and -ketoglutarate were measured with isolated rat brain mitochondria using the fluorescent H2O2 probe Amplex Red in the presence of superoxide dismutase. We hypothesized that matrix dehydrogenases, e.g., -ketoglutarate dehydrogenase complex (KGDC), are also important sources of ROS production. The sites of mitochondrial ROS production are not established but generally believed to be located within the electron transport chain. www.umm.edu /anesthesia/F_2002.html

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