Information on EC 1.1.99.39 - D-2-hydroxyglutarate dehydrogenase

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The enzyme appears in viruses and cellular organisms

EC NUMBER
COMMENTARY hide
1.1.99.39
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RECOMMENDED NAME
GeneOntology No.
D-2-hydroxyglutarate dehydrogenase
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REACTION
REACTION DIAGRAM
COMMENTARY hide
ORGANISM
UNIPROT
LITERATURE
(R)-2-hydroxyglutarate + acceptor = 2-oxoglutarate + reduced acceptor
show the reaction diagram
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SYSTEMATIC NAME
IUBMB Comments
(R)-2-hydroxyglutarate:acceptor 2-oxidoreductase
Contains FAD. The enzyme has no activity with NAD+ or NADP+, and was assayed in vitro using artificial electron acceptors. It has lower activity with (R)-lactate, (R)-2-hydroxybutyrate and meso-tartrate, and no activity with the (S) isomers. The mammalian enzyme is stimulated by Zn2+, Co2+ and Mn2+.
ORGANISM
COMMENTARY hide
LITERATURE
UNIPROT
SEQUENCE DB
SOURCE
GENERAL INFORMATION
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
metabolism
the enzyme elevates 2-oxoglutarate levels, influencing histone and DNA methylation, and HIF1alpha hydroxylation, and induces mitochondrial isocitrate dehydrogenase activity
SUBSTRATE
PRODUCT                       
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate) hide
LITERATURE
(Substrate)
COMMENTARY
(Product) hide
LITERATURE
(Product)
Reversibility
r=reversible
ir=irreversible
?=not specified
(R)-2-hydroxyglutarate + acceptor
2-oxoglutarate + reduced acceptor
show the reaction diagram
(R)-2-hydroxyglutarate + oxidized 2,6-dichlorophenolindophenol
2-oxoglutarate + reduced 2,6-dichlorophenolindophenol
show the reaction diagram
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?
additional information
?
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NATURAL SUBSTRATES
NATURAL PRODUCTS
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate) hide
LITERATURE
(Substrate)
COMMENTARY
(Product) hide
LITERATURE
(Product)
REVERSIBILITY
r=reversible
ir=irreversible
?=not specified
(R)-2-hydroxyglutarate + acceptor
2-oxoglutarate + reduced acceptor
show the reaction diagram
additional information
?
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description of two pathogenic mutations in the D-2-hydroxyglutarate dehydrogenase gene causing D-2-HGA with a very mild clinical presentation: a splice error (IVS4-2A->G) and a missense mutation (c.1315A->G, p.Asn439Asp). Mutations in the D-2-hydroxyglutarate dehydrogenase gene cause bith the severe and mild phenotypes of D-2-HGA
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COFACTOR
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
additional information
no reaction with NAD+, NADP+ or cytochrome c
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KM VALUE [mM]
SUBSTRATE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
0.584
(R)-2-hydroxyglutarate
25°C, pH 8.75, cofactor oxidized 2,6-dichlorophenolindophenol
TURNOVER NUMBER [1/s]
SUBSTRATE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
0.8
(R)-2-hydroxyglutarate
25°C, pH 8.75, cofactor oxidized 2,6-dichlorophenolindophenol
kcat/KM VALUE [1/mMs-1]
SUBSTRATE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
1.37
(R)-2-hydroxyglutarate
25°C, pH 8.75, cofactor oxidized 2,6-dichlorophenolindophenol
pH OPTIMUM
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
SOURCE TISSUE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
SOURCE
LOCALIZATION
ORGANISM
UNIPROT
COMMENTARY hide
GeneOntology No.
LITERATURE
SOURCE
MOLECULAR WEIGHT
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
61450
gel filtration
Cloned/COMMENTARY
ORGANISM
UNIPROT
LITERATURE
expressed in Escherichia coli BLR cells
ENGINEERING
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
A208T
inactive
A426T
inactive
G131X
inactive
R212W
inactive
R421H
inactive