Просмотр кода страницы Английская Википедия:D-2-hydroxyglutarate dehydrogenase

{{Английская Википедия/Панель перехода}}
{{enzyme
| Name = D-2-hydroxyglutarate dehydrogenase
| EC_number = 1.1.99.39
| CAS_number = 
| IUBMB_EC_number = 
| GO_code = 
| image =
| width =
| caption =
}}
In [[enzymology]], a '''D-2-hydroxyglutarate dehydrogenase''' ({{EC number|1.1.99.39}}) is an [[enzyme]] that [[catalysis|catalyzes]] the [[chemical reaction]]

:(R)-2-hydroxyglutarate + acceptor <math>\rightleftharpoons</math> 2-oxoglutarate + reduced acceptor

Thus, the two [[substrate (biochemistry)|substrates]] of this enzyme are [[alpha-Hydroxyglutaric acid|(R)-2-hydroxyglutarate]] and [[Electron acceptor|acceptor]], whereas its two [[product (chemistry)|products]] are [[2-oxoglutarate]] and [[reduced acceptor]].

The enzyme activity has been confirmed in animals<ref name="pmid15070399">{{cite journal | vauthors = 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> 
as well as in plants
.<ref name="pmid19586914">{{cite journal | vauthors = 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 | doi-access = free }}</ref>

== Nomenclature ==

This enzyme belongs to the family of [[oxidoreductase]]s, specifically those acting on the CH-OH group of donor with other acceptors.  The [[List of enzymes|systematic name]] of this enzyme class is '''(R)-2-hydroxyglutarate:acceptor 2-oxidoreductase'''. Other names in common use include:

{{div col|colwidth=26em}}
* (R)-2-hydroxyglutarate:(acceptor) 2-oxidoreductase
* alpha-hydroxyglutarate dehydrogenase
* alpha-hydroxyglutarate dehydrogenase (NAD{{+}} specific)
* alpha-hydroxyglutarate oxidoreductase
* alpha-ketoglutarate reductase
* hydroxyglutaric dehydrogenase
* D-alpha-hydroxyglutarate dehydrogenase
* D-alpha-hydroxyglutarate:NAD{{+}} 2-oxidoreductase
{{Div col end}}

== Clinical significance ==

Deficiency in this enzyme in humans ([[D2HGDH]]) or in the model plant ''[[Arabidopsis thaliana]]'' (At4g36400) 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 | vauthors = 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 | pmc = 2899879 }}</ref><ref name="pmid21296880">{{cite journal | vauthors = 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 | doi-access = free }}</ref>

==See also==
* [[D2HGDH]]
* [[L2HGDH]]
* [[L-2-hydroxyglutarate dehydrogenase]]
* [[2-hydroxyglutarate synthase]]
* [[Alpha-Hydroxyglutaric acid]]
* [[2-Hydroxyglutaric aciduria]]
* [[Hydroxyacid-oxoacid transhydrogenase]]

==References==
{{reflist}}

== Further reading ==
{{refbegin}}
* {{cite journal |last1=Maurino |first1=Veronica |last2=Engqvist |first2=Martin |year=2015 |title=2-Hydroxy Acids in Plant Metabolism |journal=The Arabidopsis Book |volume=13 |pages=e0182 |doi=10.1199/tab.0182|pmid=26380567 |pmc=4568905 }}
{{refend}}

{{Alcohol oxidoreductases}}
{{Enzymes}}
{{Portal bar|Biology|border=no}}

[[Category:EC 1.1.99]]
[[Category:Enzymes of unknown structure]]


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