1.1.99.39: D-2-hydroxyglutarate dehydrogenase
This is an abbreviated version!
For detailed information about D-2-hydroxyglutarate dehydrogenase, go to the full flat file.
Word Map on EC 1.1.99.39
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1.1.99.39
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acidurias
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neurometabolic
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isocitrate
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2-ketoglutarate
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l2hgdh
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l-2-hga
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d-lactate
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d-malate
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chondromatosis
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metaphyseal
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oncometabolite
- 1.1.99.39
-
acidurias
-
neurometabolic
- isocitrate
- 2-ketoglutarate
- l2hgdh
-
l-2-hga
- d-lactate
- d-malate
- chondromatosis
-
metaphyseal
-
oncometabolite
Reaction
Synonyms
2-hydroxyglutarate dehydrogenase, AtD-2HGDH, D-2-HG dehydrogenase, D-2-HGA, D-2-hydroxyglutarate dehydrogenase, D2-hydroxyglutarate dehydrogenase, D2HDH, D2HGDH
ECTree
1.1.99.39 D-2-hydroxyglutarate dehydrogenaseAdvanced search results
results (
results (Substrates Products
Substrates Products on EC 1.1.99.39 - D-2-hydroxyglutarate dehydrogenase
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SUBSTRATE 
PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate)
(Substrate)
LITERATURE
(Substrate)
(Substrate)
COMMENTARY
(Product)
(Product)
LITERATURE
(Product)
(Product)
Reversibility
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
(R)-2-hydroxyglutarate + electron transfer flavoprotein
2-oxoglutarate + reduced electron transfer flavoprotein
(R)-2-hydroxyglutarate + oxidized 2,6-dichlorophenolindophenol
2-oxoglutarate + reduced 2,6-dichlorophenolindophenol
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-
-
?
(R)-2-hydroxyglutarate + phenazine methosulfate
2-oxoglutarate + reduced phenazine methosulfate
(R)-2-hydroxyglutarate + phenazinemethosulfate
2-oxoglutarate + reduced phenazinemethosulfate
(S)-2-hydroxyglutarate + acceptor
2-oxoglutarate + reduced acceptor
necessary step in the serine biosynthetic pathway
-
-
?
D-malate + phenazine methosulfate
? + reduced phenazine methosulfate
-
-
-
?
(R)-2-hydroxyglutarate + acceptor
2-oxoglutarate + reduced acceptor
-
-
-
-
?
(R)-2-hydroxyglutarate + acceptor
2-oxoglutarate + reduced acceptor
-
-
-
?
(R)-2-hydroxyglutarate + acceptor
2-oxoglutarate + reduced acceptor
-
-
-
?
(R)-2-hydroxyglutarate + acceptor
2-oxoglutarate + reduced acceptor
-
-
-
-
?
(R)-2-hydroxyglutarate + acceptor
2-oxoglutarate + reduced acceptor
-
-
-
-
?
(R)-2-hydroxyglutarate + cytochrome c
2-oxoglutarate + reduced cytochrome c
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-
-
-
?
(R)-2-hydroxyglutarate + cytochrome c
2-oxoglutarate + reduced cytochrome c
-
-
-
-
?
(R)-2-hydroxyglutarate + electron transfer flavoprotein
2-oxoglutarate + reduced electron transfer flavoprotein
-
-
-
?
(R)-2-hydroxyglutarate + electron transfer flavoprotein
2-oxoglutarate + reduced electron transfer flavoprotein
-
-
-
-
?
(R)-2-hydroxyglutarate + electron transfer flavoprotein
2-oxoglutarate + reduced electron transfer flavoprotein
-
-
-
-
?
(R)-2-hydroxyglutarate + phenazine methosulfate
2-oxoglutarate + reduced phenazine methosulfate
-
-
-
?
(R)-2-hydroxyglutarate + phenazine methosulfate
2-oxoglutarate + reduced phenazine methosulfate
-
-
-
?
(R)-2-hydroxyglutarate + phenazinemethosulfate
2-oxoglutarate + reduced phenazinemethosulfate
-
-
-
-
?
(R)-2-hydroxyglutarate + phenazinemethosulfate
2-oxoglutarate + reduced phenazinemethosulfate
-
-
-
-
?
additional information
?
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low activity with D-lactate, D-2-hydroxybutyrate, meso-tartrate
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-
?
additional information
?
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-
low activity with D-lactate, D-2-hydroxybutyrate, meso-tartrate
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-
?
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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-
?
