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| {{enzyme
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| | Name = 2-hydroxyglutarate dehydrogenase
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| | EC_number = 1.1.99.2
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| | CAS_number = 9028-80-2
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| | IUBMB_EC_number = 1/1/99/2
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| | GO_code = 0047545
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| | image =
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| }}
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| In [[enzymology]], a '''2-hydroxyglutarate dehydrogenase''' ({{EC number|1.1.99.2}}) is an [[enzyme]] that [[catalysis|catalyzes]] the [[chemical reaction]]
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| :(S)-2-hydroxyglutarate + acceptor <math>\rightleftharpoons</math> 2-oxoglutarate + reduced acceptor
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| Thus, the two [[substrate (biochemistry)|substrates]] of this enzyme are [[alpha-Hydroxyglutaric acid|(S)-2-hydroxyglutarate]] and [[Electron acceptor|acceptor]], whereas its two [[product (chemistry)|products]] are [[2-oxoglutarate]] and [[reduced acceptor]]. However, enzymes with specificity to (R)-2-hydroxyglutarate dehydrogenase also exist in both mammals<ref name="pmid15070399">{{cite journal | author = Achouri Y, Noël G, Vertommen D, Rider MH, Veiga-Da-Cunha M, Van Schaftingen E | title = Identification of a dehydrogenase acting on D-2-hydroxyglutarate | journal = Biochem. J. | volume = 381 | issue = Pt 1 | pages = 35–42 |date=July 2004 | pmid = 15070399 | pmc = 1133759 | doi = 10.1042/BJ20031933 }}</ref> and plants<ref name="pmid19586914">{{cite journal | author = Engqvist M, Drincovich MF, Flügge UI, Maurino VG | title = Two D-2-hydroxy-acid dehydrogenases in Arabidopsis thaliana with catalytic capacities to participate in the last reactions of the methylglyoxal and beta-oxidation pathways | journal = J. Biol. Chem. | volume = 284 | issue = 37 | pages = 25026–37 |date=September 2009 | pmid = 19586914 | pmc = 2757207 | doi = 10.1074/jbc.M109.021253 }}</ref> even though they have not been given a unique EC number. This enzyme participates in [[Butyric_acid#Biosynthesis|butanoate metabolism]].
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| == Nomenclature ==
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| This enzyme belongs to the family of [[oxidoreductase]]s, specifically those acting on the CH-OH group of donor with other acceptors. The systematic name of this enzyme class is '''(S)-2-hydroxyglutarate:acceptor 2-oxidoreductase'''. Other names in common use include:
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| {{div col|colwidth=26em}}
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| * (S)-2-hydroxyglutarate:(acceptor) 2-oxidoreductase
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| * alpha-hydroxyglutarate dehydrogenase
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| * alpha-hydroxyglutarate dehydrogenase (NAD{{+}} specific)
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| * alpha-hydroxyglutarate oxidoreductase
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| * alpha-ketoglutarate reductase
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| * hydroxyglutaric dehydrogenase
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| * L-alpha-hydroxyglutarate dehydrogenase
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| * L-alpha-hydroxyglutarate:NAD{{+}} 2-oxidoreductase
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| {{Div col end}}
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| == Clinical significance ==
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| Deficiency in this enzyme in humans ([[D2HGDH]]) or in the model plant ''[[Arabidopsis thaliana]]'' leads to massive accumulation of [[alpha-Hydroxyglutaric acid|D-2-hydroxyglutarate]]. In humans this results in the fatal neurometabolic disorder [[2-Hydroxyglutaric aciduria]] whereas plants seem to be to a large extent unaffected by high cellular concentrations of this compound.<ref name="pmid20501910">{{cite journal | author = Araújo WL, Ishizaki K, Nunes-Nesi A, Larson TR, Tohge T, Krahnert I, Witt S, Obata T, Schauer N, Graham IA, Leaver CJ, Fernie AR | title = Identification of the 2-hydroxyglutarate and isovaleryl-CoA dehydrogenases as alternative electron donors linking lysine catabolism to the electron transport chain of Arabidopsis mitochondria | journal = Plant Cell | volume = 22 | issue = 5 | pages = 1549–63 |date=May 2010 | pmid = 20501910 | doi = 10.1105/tpc.110.075630 | url = | issn = | pmc = 2899879 }}</ref><ref name="pmid21296880">{{cite journal | author = Engqvist MK, Kuhn A, Wienstroer J, Weber K, Jansen EE, Jakobs C, Weber AP, Maurino VG | title = Plant D-2-hydroxyglutarate dehydrogenase participates in the catabolism of lysine especially during senescence | journal = J Biol Chem | volume = 286 | issue = April 1 | pages = 11382–11390 |date=April 2011 | pmid = 21296880 | doi = 10.1074/jbc.M110.194175 | pmc = 3064194 }}</ref>
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| ==See also==
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| * [[L2HGDH]]
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| * [[2-hydroxyglutarate synthase]]
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| * [[Alpha-Hydroxyglutaric acid]]
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| * [[2-Hydroxyglutaric aciduria]]
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| * [[Hydroxyacid-oxoacid transhydrogenase]]
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| ==References==
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| {{reflist}}
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| == Further reading ==
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| {{refbegin}}
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| * {{cite journal | author = Weil-Malherbe H | year = 1937 | title = The oxidation of l(-)alpha-hydroxyglutaric acid in animal tissues | journal = Biochem. J. | volume = 31 | issue = 11 | pages = 2080–2094 | pmid=16746551 | pmc = 1267182}}
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| {{refend}}
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| [[Category:EC 1.1.99]]
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| [[Category:Enzymes of unknown structure]]
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| {{1.1-enzyme-stub}}
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